2019

Swainson LA, | Ahn H, | Pajanirassa P, | Khetarpal V, | Deleage C, | Estes JD, | Hunt PW, | Munoz-Sanjuan I, | McCune JM |

Kynurenine 3-Monooxygenase inhibition during acute simian immunodeficiency virus infection lowers PD-1 expression and improves post-combination antiretroviral therapy CD4(+) T cell counts and body weight

J Immunol

899-910

203

(4):

20190815

PMID:31285277

DOI: 10.4049/jimmunol.1801649

PMCID: 6684450

Federspiel JD, | Greco TM, | Lum KK, | Cristea IM |

Hdac4 interactions in Huntington's disease viewed through the prism of Multiomics

Mol Cell Proteomics

S92-S113

18

(8):

20190809

PMID:31040226

DOI: 10.1074/mcp.RA118.001253

PMCID:

GeM-HD Consortium, | Lee JM, | Correia K, | Loupe J, | Kim KH, | Barker D, | Hong EP, | Chao MJ, | Long JD, | Lucente D, | Vonsattel JPG, | Pinto RM, | Abu Elneel K, | Ramos EM, | Mysore JS, | Gillis T, | Wheeler VC, | MacDonald ME, | Gusella JF, | McAllister B, | Massey T, | Medway C, | Stone TC, | Hall L, | Jones L, | Holmans P, | Kwak S, | Ehrhardt AG, | Sampaio C, | Ciosi M, | Maxwell A, | Chatzi A, | Monckton DG, | Orth M, | Landwehrmeyer GB, | Paulsen JS, | Dorsey ER, | Shoulson I, | Myers RH |

CAG repeat not polyglutamine length determines timing of Huntington's disease onset

Cell

887-900

178

(4):

20190808

PMID:31398342

DOI: 10.1016/j.cell.2019.06.036

PMCID:

Alterman JF, | Godinho BMDC, | Hassler MR, | Ferguson CM, | Echeverria D, | Sapp E, | Haraszti RA, | Coles AH, | Conroy F, | Miller R, | Roux L, | Yan P, | Knox EG, | Turanov AA, | King RM, | Gernoux G, | Mueller C, | Gray-Edwards HL, | Moser RP, | Bishop NC, | Jaber SM, | Gounis MJ, | Sena-Esteves M, | Pai AA, | DiFiglia M, | Aronin N, | Khvorova A |

A divalent siRNA chemical scaffold for potent and sustained modulation of gene expression throughout the central nervous system

Nat Biotechnol

884-894

37

(8):

20190802

PMID:31375812

DOI: 10.1038/s41587-019-0205-0

PMCID:

Kacher R, | Lamazière A, | Heck N, | Kappes V, | Mounier C, | Despres G, | Dembitskaya Y, | Perrin E, | Christaller W, | Sasidharan Nair S, | Messent V, | Cartier N, | Vanhoutte P, | Venance L, | Saudou F, | Néri C, | Caboche J, | Betuing S |

CYP46A1 gene therapy deciphers the role of brain cholesterol metabolism in Huntington's disease

Brain

2432-2450

142

(8):

20190801

PMID:31286142

DOI: 10.1093/brain/awz174

PMCID:

Flower M, | Lomeikaite V, | Ciosi M, | Cumming S, | Morales F, | Lo K, | Hensman Moss D, | Jones L, | Holmans P, | TRACK-HD Investigators, | OPTIMISTIC Consortium, | Monckton DG, | Tabrizi SJ |

MSH3 modifies somatic instability and disease severity in Huntington's and myotonic dystrophy type 1

Brain

1876–1886

142

(7):

20190715

PMID:31216018

DOI: 10.1093/brain/awz115

PMCID: 6598626

Wilkes FA, | Abaryan Z, | Ching CRK, | Gutman BA, | Madsen SK, | Walterfang M, | Velakoulis D, | Stout JC, | Chua P, | Egan GF, | Thompson PM, | Looi JCL, | Georgiou-Karistianis N |

Striatal morphology and neurocognitive dysfunction in Huntington disease: the IMAGE-HD study

Psychiatry Res Neuroimaging

1-8

291

():

20190712

PMID:31330407

DOI: 10.1016/j.pscychresns.2019.07.003

PMCID:

Franich NR, | Hickey MA, | Zhu C, | Osborne GF, | Ali N, | Chu T, | Bove NH, | Lemesre V, | Lerner RP, | Zeitlin SO, | Howland D, | Neueder A, | Landles C, | Bates GP, | Chesselet MF |

Phenotype onset in Huntington's disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene

J Neurosci Res

():

20190708

PMID:31282030

DOI: 10.1002/jnr.24493

PMCID:

Zeitler B, | Froelich S, | Marlen K, | Shivak DA, | Yu Q, | Li D, | Pearl JR, | Miller JC, | Zhang L, | Paschon DE, | Hinkley SJ, | Ankoudinova I, | Lam S, | Guschin D, | Kopan L, | Cherone JM, | Nguyen HB, | Qiao G, | Ataei Y, | Mendel MC, | Amora R, | Surosky R, | Laganiere J, | Vu BJ, | Narayanan A, | Sedaghat Y, | Tillack K, | Thiede C, | Gärtner A, | Kwak S, | Bard J, | Mrzljak L, | Park L, | Heikkinen T, | Lehtimäki KK, | Svedberg MM, | Häggkvist J, | Tari L, | Tóth M, | Varrone A, | Halldin C, | Kudwa AE, | Ramboz S, | Day M, | Kondapalli J, | Surmeier DJ, | Urnov FD, | Gregory PD, | Rebar EJ, | Muñoz-Sanjuán I, | Zhang HS |

Allele-selective transcriptional repression of mutant HTT for the treatment of Huntington's disease

Nat Med

1131-1142

25

(7):

20190701

PMID:31263285

DOI: 10.1038/s41591-019-0478-3

PMCID:

Bigan E, | Sasidharan Nair S, | Lejeune FX, | Fragnaud H, | Parmentier F, | Mégret L, | Verny M, | Aaronson J, | Rosinski J, | Neri C |

Genetic cooperativity in multi-layer networks implicates cell survival and senescence in the striatum of Huntington's disease mice synchronous to symptoms

Bioinformatics

():

20190624

PMID:31228193

DOI: 10.1093/bioinformatics/btz514

PMCID:

Poudel GR, | Harding IH, | Egan GF, | Georgiou-Karistianis N |

Network spread determines severity of degeneration and disconnection in Huntington's disease

Hum Brain Mapp

1-10

():

20190612

PMID:31187915

DOI: 10.1002/hbm.24695

PMCID:

Huguet G, | Temel Y, | Kádár E, | Pol S, | Casaca-Carreira J, | Segura-Torres P, | Jahanshahi A |

Altered expression of dopaminergic cell fate regulating genes prior to manifestation of symptoms in a transgenic rat model of Huntington's disease

Brain Res

101-108

1712

():

20190601

PMID:30711400

DOI: 10.1016/j.brainres.2019.01.041

PMCID:

Ochaba J, | Fote G, | Kachemov M, | Thein S, | Yeung SY, | Lau AL, | Hernandez S, | Lim RG, | Casale M, | Neel MJ, | Monuki ES, | Reidling J, | Housman DE, | Thompson LM, | Steffan JS |

IKKβ slows Huntington's disease progression in R6/1 mice

Proc Natl Acad Sci USA

10952-10961

116

(22):

20190528

PMID:31088970

DOI: 10.1073/pnas.1814246116

PMCID:

Octeau JC, | Gangwani MR, | Allam SL, | Tran D, | Huang S, | Hoang-Trong TM, | Golshani P, | Rumbell TH, | Kozloski JR, | Khakh BS |

Transient, consequential increases in extracellular potassium ions accompany Channelrhodopsin2 excitation

Cell Rep

2249-2261.e7

27

(8):

20190521

PMID:31116972

DOI: 10.1016/j.celrep.2019.04.078

PMCID: 6582980

Dvorzhak A, | Helassa N, | Török K, | Schmitz D, | Grantyna R |

Single synapse indicators of impaired glutamate clearance derived from fast iGlu u imaging of cortical afferents in the striatum of normal and Huntington (Q175) mice

J Neurosci

3970-3982

39

(20):

20190515

PMID:30819797

DOI: 10.1523/JNEUROSCI.2865-18.2019

PMCID:

Svedberg MM, | Varnäs K, | Varrone A, | Mitsios N, | Mulder J, | Gulyás B, | Beaumont V, | Munoz-Sanjuan I, | Zaleska MM, | Schmidt CJ, | Halldin C, | Mrzljak L |

In vitro phosphodiesterase 10A (PDE10A) binding in whole hemisphere human brain using the PET radioligand [(18)F] MNI-659

Brain Res

140-145

1711

():

20190515

PMID:30664847

DOI: 10.1016/j.brainres.2019.01.021

PMCID:

Holley SM, | Galvan L, | Kamdjou T, | Dong A, | Levine MS, | Cepeda C |

Major contribution of somatostatin-expressing interneurons and cannabinoid receptors to increased GABA synaptic activity in the striatum of Huntington's disease mice

Front Synaptic Neurosci

11

(14):

20190514

PMID:31139071

DOI: 10.3389/fnsyn.2019.00014

PMCID: 6527892

Sharma M, | Subramaniam S |

Rhes travels from cell to cell and transports Huntington disease protein via TNT-like protrusion

J Cell Biol

pii: jcb.201807068

():

20190510

PMID:31076452

DOI: 10.1083/jcb.201807068

PMCID:

Pido-Lopez J, | Tanudjojo B, | Farag S, | Bondulich MK, | Andre R, | Tabrizi SJ, | Bates GP |

Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington's disease by etanercept treatment

Sci Rep

7202

9

(1):

20190510

PMID:31076648

DOI: 10.1038/s41598-019-43627-3

PMCID: 6510744

Di Marco A, | Gonzalez Paz O, | Fini I, | Vignone D, | Cellucci A, | Battista MR, | Auciello G, | Orsatti L, | Zini M, | Monteagudo E, | Khetarpal V, | Rose M, | Dominguez C, | Herbst T, | Toledo-Sherman L, | Summa V, | Muñoz-Sanjuán I |

Application of an in vitro blood-brain barrier model in the selection of experimental drug candidates for the treatment of Huntington's disease

Mol Pharm

2069-2082

16

(5):

20190506

PMID:30916978

DOI: 10.1021/acs.molpharmaceut.9b00042

PMCID:

Shoulson I, | Eberly S, | Oakes D, | Kayson E, | Young AB | PHAROS Investigators |

Phenotype-genotype discrepancies in the prospective Huntington at-risk observational study

Ann Clin Transl Neurol

1046-1052

6

(6):

20190503

PMID:31211168

DOI: 10.1002/acn3.781

PMCID: 6562068

Vizcarra JA, | Sánchez-Ferro Á, | Maetzler W, | Marsili L, | Zavala L, | Lang AE, | Martinez-Martin P, | Mestre TA, | Reilmann R, | Hausdorff JM, | Dorsey ER, | Paul SS, | Dexheimer JW, | Wissel BD, | Fuller RLM, | Bonato P, | Tan AH, | Bloem BR, | Kopil C, | Daeschler M, | Bataille L, | Kleiner G, | Cedarbaum JM, | Klucken J, | Merola A, | Goetz CG, | Stebbins GT, | Espay AJ | MDS Technology Task Force and | MDS Rating Scales Program Electronic Development Ad-Hoc Committee |

The Parkinson's disease e-diary: Developing a clinical and research tool for the digital age

Mov Disord

676-681

34

(5):

20190501

PMID:30901492

DOI: 10.1002/mds.27673

PMCID:

Kojer K, | Hering T, | Bazenet C, | Weiss A, | Herrmann F, | Taanman JW, | Orth M |

Huntingtin aggregates and mitochondrial pathology in skeletal muscle but not heart of late-stage R6/2 mice

J Huntingtons Dis

145–159

8

(2):

20190430

PMID:30814364

DOI: 10.3233/JHD-180324

PMCID:

Harding RJ, | Loppnau P, | Ackloo S, | Lemak A, | Hutchinson A, | Hunt B, | Holehouse AS, | Ho JC, | Fan L, | Toledo-Sherman L, | Seitova A, | Arrowsmith CH |

Design and characterization of mutant and wildtype huntingtin proteins produced from a toolkit of scalable eukaryotic expression systems

J Biol Chem

6986-7001

294

(17):

20190426

PMID:30842263

DOI: 10.1074/jbc.RA118.007204

PMCID:

Carrillo-Reid L, | Day M, | Xie Z, | Melendez AE, | Kondapalli J, | Plotkin JL, | Wokosin DL, | Chen Y, | Kress GJ, | Kaplitt M, | Ilijic E, | Guzman JN, | Chan SS, | Surmeier DJ |

Mutant huntingtin enhances activation of dendritic Kv4 K(+) channels in striatal spiny projection neurons

Elife

e40818

8

():

20190424

PMID:31017573

DOI: 10.7554/eLife.40818

PMCID:

Espinoza FA, | Liu J, | Ciarochi J, | Turner JA, | Vergara VM, | Caprihan A, | Misiura M, | Johnson HJ, | Long JD, | Bockholt JH, | Paulsen JS, | Calhoun VD |

Dynamic functional network connectivity in Huntington's disease and its associations with motor and cognitive measures

Hum Brain Mapp

1955-1968

40

(6):

20190415

PMID:30618191

DOI: 10.1002/hbm.24504

PMCID:

Todorov DI, | Capps RA, | Barnett WH, | Latash EM, | Kim T, | Hamade KC, | Markin SN, | Rybak IA, | Molkov YI |

The interplay between cerebellum and basal ganglia in motor adaptation: A modeling study

PLoS One

e0214926

14

(4):

20190412

PMID:30978216

DOI: 10.1371/journal.pone.0214926

PMCID: 6461234

Tang X, | Ross CA, | Johnson H, | Paulsen JS, | Younes L, | Albin RL, | Ratnanather JT, | Miller MI |

Regional subcortical shape analysis in premanifest Huntington's disease

Hum Brain Mapp

1419-1433

40

(5):

20190401

PMID:30376191

DOI: 10.1002/hbm.24456

PMCID:

Toledo-Sherman L, | Breccia P, | Cachope R, | Bate JR, | Angulo-Herrera I, | Wishart G, | Matthews KL, | Martin SL, | Cox HC, | McAllister G, | Penrose SD, | Vater H, | Esmieu W, | Van de Poël A, | Van de Bospoort R, | Strijbosch A, | Lamers M, | Leonard P, | Jarvis RE, | Blackaby W, | Barnes K, | Eznarriaga M, | Dowler S, | Smith GD, | Fischer DF, | Lazari O, | Yates D, | Rose M, | Jang SW, | Muñoz-Sanjuan I, | Dominguez C |

Optimization of potent and selective ataxia telangiectasia-mutated inhibitors suitable for a proof-of-concept study in Huntington's disease models

J Med Chem

2988-3008

62

(6):

20190328

PMID:30840447

DOI: 10.1021/acs.jmedchem.8b01819

PMCID:

Reindl W, Baldo B, Schulz J, Janack I, Lindner I, Kleinschmidt M, | Sedaghat Y, | Thiede C, | Tillack K, | Schmidt C, | Cardaun I, | Schwagarus T, | Herrmann F, | Hotze M, | Osborne GF, | Herrmann S, | Weiss A, | Zerbinatti C, | Bates GP, | Bard J, | Munoz-Sanjuan I, | Macdonald D |

Meso scale discovery-based assays for the detection of aggregated huntingtin

PLoS One

e0213521

14

(3):

20190326

PMID:30913220

DOI: 10.1371/journal.pone.0213521

PMCID:

Murthy V, | Tebaldi T, | Yoshida T, | Erdin S, | Calzonetti T, | Vijayvargia R, | Tripathi T, | Kerschbamer E, | Seong IS, | Quattrone A, | Talkowski ME, | Gusella JF, | Georgopoulos K, | MacDonald ME, | Biagioli M |

Hypomorphic mutation of the mouse Huntington's disease gene orthologue

PLoS Genet

e1007765

15

(3):

20190321

PMID:30897080

DOI: 10.1371/journal.pgen.1007765

PMCID:

Merienne N, | Meunier C, | Schneider A, | Seguin J, | Nair SS, | Rocher AB, | Le Gras S, | Keime C, | Faull R, | Pellerin L, | Chatton JY, | Neri C, | Merienne K, | Déglon N |

Cell-type-specific gene expression profiling in adult mouse brain reveals normal and disease-state signatures

Cell Rep

2477-2493

26

(9):

20190226

PMID:30811995

DOI: 10.1016/j.celrep.2019.02.003

PMCID:

Castaldo I, | De Rosa M, | Romano A, | Zuchegna C, | Squitieri F, | Mechelli R, | Peluso S, | Borrelli C, | Del Mondo A, | Salvatore E, | Vescovi LA, | Migliore S, | De Michele G, | Ristori G, | Romano S, | Avvedimento EV, | Porcellini A |

DNA damage signatures in peripheral blood cells as biomarkers in prodromal huntington disease

Ann Neurol

296-301

85

(2):

20190221

PMID:30549309

DOI: 10.1002/ana.25393

PMCID:

Kang R, | Wang L, | Sanders SS, | Zuo K, | Hayden MR, | Raymond LA |

Altered regulation of striatal neuronal N-Methyl-D-Aspartate receptor trafficking by palmitoylation in Huntington disease mouse model

Front Synaptic Neurosci

11

(3):

20190221

PMID:30846936

DOI: 10.3389/fnsyn.2019.00003

PMCID: 6393405

Goold R, | Flower M, | Moss DH, | Medway C, | Wood-Kaczmar A, | Andre R, | Farshim P, | Bates GP, | Holmans P, | Jones L, | Tabrizi SJ |

FAN1 modifies Huntington's disease progression by stabilizing the expanded HTT CAG repeat

Hum Mol Genet

650-661

28

(4):

20190215

PMID:30358836

DOI: 10.1093/hmg/ddy375

PMCID: 6360275

Gatto RG, | Ye AQ, | Colon-Perez L, | Mareci TH, | Lysakowski A, | Price SD, | Brady ST, | Karaman M, | Morfini G, | Magin RL |

Detection of axonal degeneration in a mouse model of Huntington's disease: comparison between diffusion tensor imaging and anomalous diffusion metrics

MAGMA

():

20190215

PMID:30771034

DOI: 10.1007/s10334-019-00742-6

PMCID:

Tousley A, | Iuliano M, | Weisman E, | Sapp E, | Richardson H, | Vodicka P, | Alexander J, | Aronin N, | DiFiglia M, | Kegel-Gleason KB |

Huntingtin associates with the actin cytoskeleton and α-actinin isoforms to influence stimulus dependent morphology changes

PLoS One

e0212337

14

(2):

20190215

PMID:30768638

DOI: 10.1371/journal.pone.0212337

PMCID:

Smatlikova P, | Juhas S, | Juhasova J, | Suchy T, | Hubalek Kalbacova M, | Ellederova Z, | Motlik J, | Klima J |

Adipogenic differentiation of bone marrow-derived mesenchymal stem cells in pig transgenic model expressing human mutant huntingtin

J Huntingtons Dis

33-51

8

(1):

20190213

PMID:30584151

DOI: 10.3233/JHD-180303

PMCID:

Rieke L, | Fels M, | Schubert R, | Habbel B, | Matheis T, | Schuldenzucker V, | Kemper N, | Reilmann R |

Activity behaviour of minipigs transgenic for the Huntington gene

J Huntingtons Dis

23-31

8

(1):

20190213

PMID:30689591

DOI: 10.3233/JHD-180325

PMCID:

Perez-Rosello T, | Gelman S, | Tombaugh G, | Cachope R, | Beaumont V, | Surmeier DJ |

Enhanced striatopallidal gamma-aminobutyric acid (GABA)(A) receptor transmission in mouse models of huntington's disease

Mov Disord

684-696

34

(5):

20190206

PMID:30726572

DOI: 10.1002/mds.27622

PMCID:

Ciarochi JA, | Johnson HJ, | Calhoun VD, | Liu J, | Espinoza FA, | Bockholt HJ, | Misiura M, | Caprihan A, | Plis S, | Paulsen JS, | Turner JA, | PREDICT-HD Investigators and Coordinators of the Huntington Study Group |

Concurrent cross-sectional and longitudinal analyses of multivariate white matter profiles and clinical functioning in pre-diagnosis Huntington disease

J Huntingtons Dis

199-219

8

(2):

20190205

PMID:30932891

DOI: 10.3233/JHD-180332

PMCID:

Padovan-Neto FE, | Jurkowski L, | Murray C, | Stutzmann GE, | Kwan M, | Ghavami A, | Beaumont V, | Park LC, | West AR |

Age- and sex-related changes in cortical and striatal nitric oxide synthase in the Q175 mouse model of Huntington's disease

Nitric Oxide

40-50

83

():

20190201

PMID:30528913

DOI: 10.1016/j.niox.2018.12.002

PMCID:

Paulsen JS, | Lourens S, | Kieburtz K, | Zhang Y |

Sample enrichment for clinical trials to show delay of onset in Huntington disease

Mov Disord

274-280

34

(2):

20190201

PMID:30644132

DOI: 10.1002/mds.27595

PMCID:

Holley SM, | Galvan L, | Kamdjou T, | Cepeda C, | Levine MS |

Striatal GABAergic interneuron dysfunction in the Q175 mouse model of Huntington's disease

Eur J Neurosci

79-93

49

(1):

20190130

PMID:30472747

DOI: 10.1111/ejn.14283

PMCID:

Rodrigues FB, | Byrne LM, | De Vita E, | Johnson EB, | Hobbs NZ, | Thornton JS, | Scahill RI, | Wild EJ |

Cerebrospinal fluid flow dynamics in Huntington's disease evaluated by phase contrast MRI

Eur J Neurosci

1-8

():

20190128

PMID:30687961

DOI: 10.1111/ejn.14356

PMCID:

Lu M, | Banetta L, | Young LJ, | Smith EJ, | Bates GP, | Zaccone A, | Kaminski Schierle GS, | Tunnacliffe A, | Kaminski CF |

Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assembly

J Biol Chem

257-268

294

(1):

20190104

PMID:30401748

DOI: 10.1074/jbc.RA118.003500

PMCID: 6322900

Osipovitch M, | Asenjo Martinez A, | Mariani JN, | Cornwell A, | Dhaliwal S, | Zou L, | Chandler-Militello D, | Wang S, | Li X, | Benraiss SJ, | Agate R, | Lampp A, | Benraiss A, | Windrem MS, | Goldman SA |

Human ESC-derived chimeric mouse models of Huntington's disease reveal cell-intrinsic defects in glial progenitor cell differentiation

Cell Stem Cell

107-122

24

(1):

20190103

PMID:30554964

DOI: 10.1016/j.stem.2018.11.010

PMCID:

Luckhurst CA, | Aziz O, | Beaumont V, | Bürli RW, | Breccia P, | Maillard MC, | Haughan AF, | Lamers M, | Leonard P, | Matthews KL, | Raphy G, | Stott AJ, | Munoz-Sanjuan I, | Thomas B, | Wall M, | Wishart G, | Yates D, | Dominguez C |

Development and characterization of a CNS-penetrant benzhydryl hydroxamic acid class IIa histone deacetylase inhibitor

Bioorg Med Chem Lett

83-88

29

(1):

20190101

PMID:30463802

DOI: 10.1016/j.bmcl.2018.11.009

PMCID:

2018

Bravo-Arredondo JM, | Kegulian NC, | Schmidt T, | Pandey NK, | Situ AJ, | Ulmer TS, | Langen R |

The folding equilibrium of huntingtin exon 1 monomer depends on its polyglutamine tract

J Biol Chem

19613-19623

293

(51):

20181221

PMID:30315108

DOI: 10.1074/jbc.RA118.004808

PMCID: 6314148

Naze S, | Humble J, | Zheng P, | Barton S, | Rangel-Barajas C, | Rebec GV, | Kozloski JR |

Cortico-striatal cross-frequency coupling and gamma genesis disruptions in Huntington's disease mouse and computational models

eNeuro

5

(6):

20181221

PMID:30627632

DOI: 10.1523/ENEURO.0210-18.2018

PMCID: 6325534

Wang D, | Zhong L, | Li M, | Li J, | Tran K, | Ren L, | He R, | Xie J, | Moser RP, | Fraser C, | Kuchel T, | Sena-Esteves M, | Flotte TR, | Aronin N, | Gao G |

Adeno-associated virus neutralizing antibodies in large animals and their impact on brain intraparenchymal gene transfer

Mol Ther Methods Clin Dev

65-72

11

():

20181214

PMID:30397628

DOI: 10.1016/j.omtm.2018.09.003

PMCID: 6205343

McBride SD, | Morton AJ |

Indices of comparative cognition: assessing animal models of human brain function

Exp Brain Res

3379-3390

236

(12):

20181203

PMID:30267138

DOI: 10.1007/s00221-018-5370-8

PMCID: 6267686

Kuljis D, | Kudo T, | Tahara Y, | Ghiani CA, | Colwell CS |

Pathophysiology in the suprachiasmatic nucleus in mouse models of Huntington's disease

J Neurosci Res

1862-1875

96

(12):

20181203

PMID:30168855

DOI: 10.1002/jnr.24320

PMCID:

Mondo E, | Moser R, | Gao G, | Mueller C, | Sena-Esteves M, | Sapp E, | Pfister E, | O'Connell D, | Takle K, | Erger KE, | Liu W, | Conlon TJ, | DiFiglia M, | Gounis MJ, | Aronin N |

Selective neuronal uptake and distribution of AAVrh8, AAV9, and AAVrh10 in sheep after intra-striatal administration

J Huntingtons Dis

309-319

7

(4):

20181124

PMID:30320596

DOI: 10.3233/JHD-180302

PMCID:

Garcia TP, | Wang Y, | Shoulson I, | Paulsen JS, | Marder K |

Disease progression in Huntington disease: an analysis of multiple longitudinal outcomes

J Huntingtons Dis

337-344

7

(4):

20181123

PMID:30400103

DOI: 10.3233/JHD-180297

PMCID:

Long JD, | Mills JA |

Joint modeling of multivariate longitudinal data and survival data in several observational studies of Huntington's disease

BMC Med Res Methodol

138

18

(1):

20181116

PMID:30445915

DOI: 10.1186/s12874-018-0592-9

PMCID: 6240282

Godinho BMDC, | Henninger N, | Bouley J, | Alterman JF, | Haraszti RA, | Gilbert JW, | Sapp E, | Coles AH, | Biscans A, | Nikan M, | Echeverria D, | DiFiglia M, | Aronin N, | Khvorova A |

Transvascular delivery of hydrophobically modified siRNAs: gene silencing in the rat brain upon disruption of the blood-brain barrier

Mol Ther

2580-2591

26

(11):

20181107

PMID:30143435

DOI: 10.1016/j.ymthe.2018.08.005

PMCID:

Rocha NP, | Mwangi B, | Gutierrez Candano CA, | Sampaio C, | Furr Stimming E, | Teixeira AL |

The clinical picture of psychosis in manifest Huntington's disease: a comprehensive analysis of the Enroll-HD database

Front Neurol

9

(930):

20181106

PMID:30459704

DOI: 10.3389/fneur.2018.00930

PMCID: 6232301

Rowley CD, | Tabrizi SJ, | Scahill RI, | Leavitt BR, | Roos RAC, | Durr A, | Bock NA |

Altered intracortical T(1)-weighted/T(2)-weighted ratio signal in Huntington's disease

Front Neurosci

805

12

():

20181105

PMID:30455625

DOI: 10.3389/fnins.2018.00805

PMCID: 6230564

Zhang J, | Gregory S, | Scahill RI, | Durr A, | Thomas DL, | Lehericy S, | Rees G, | Tabrizi SJ, | Zhang H, | TrackOn-HD investigators |

In vivo characterization of white matter pathology in premanifest huntington's disease

Ann Neurol

497-504

84

(4):

20181029

PMID:30063250

DOI: 10.1002/ana.25309

PMCID: 6221120

Bertoglio D, | Verhaeghe J, | Kosten L, | Thomae D, | Van der Linden A, | Stroobants S, | Wityak J, | Dominguez C, | Mrzljak L, | Staelens S |

MR-based spatial normalization improves [18F]MNI-659 PET regional quantification and detectability of disease effect in the Q175 mouse model of Huntington's disease

PLoS One

e0206613

13

(10):

20181026

PMID:30365550

DOI: 10.1371/journal.pone.0206613

PMCID: 6203386

Piiponniemi TO, | Parkkari T, | Heikkinen T, | Puoliväli J, | Park LC, | Cachope R, | Kopanitsa MV |

Impaired performance of the Q175 mouse model of Huntington's disease in the touch screen paired associates learning task

Front Behav Neurosci

12

(226):

20181002

PMID:30333735

DOI: 10.3389/fnbeh.2018.00226

PMCID: 6176131

Aron R, | Pellegrini P, | Green EW, | Maddison DC, | Opoku-Nsiah K, | Wong JS, | Daub AC, | Giorgini F, | Finkbeiner S |

Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease

Nat Commun

3191

9

(1):

20180928

PMID:30266909

DOI: 10.1038/s41467-018-05653-z

PMCID: 6162324

Neueder A, | Dumas AA, | Benjamin AC, | Bates GP |

Regulatory mechanisms of incomplete huntingtin mRNA splicing

Nat Commun

3955

9

(1):

20180927

PMID:30262848

DOI: 10.1038/s41467-018-06281-3

PMCID: 6160442

Byrne LM, | Rodrigues FB, | Johnson EB, | Wijeratne PA, | De Vita E, | Alexander DC, | Palermo G, | Czech C, | Schobel S, | Scahill RI, | Heslegrave A, | Zetterberg H, | Wild EJ |

Evaluation of mutant huntingtin and neurofilament proteins as potential markers in Huntington's disease

Sci Transl Med

eaat7108

10

(458):

20180912

PMID:30209243

DOI: 10.1126/scitranslmed.aat7108

PMCID:

Siebzehnrübl FA, | Raber KA, | Urbach YK, | Schulze-Krebs A, | Canneva F, | Moceri S, | Habermeyer J, | Achoui D, | Gupta B, | Steindler DA, | Stephan M, | Nguyen HP, | Bonin M, | Riess O, | Bauer A, | Aigner L, | Couillard-Despres S, | Paucar MA, | Svenningsson P, | Osmand A, | Andreew A, | Zabel C, | Weiss A, | Kuhn R, | Moussaoui S, | Blockx I, | Van der Linden A, | Cheong RY, | Roybon L, | Petersén Å, | von Hörsten S |

Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition

Proc Natl Acad Sci USA

E8765-E8774

115

(37):

20180911

PMID:30150378

DOI: 10.1073/pnas.1807962115

PMCID: 6140493

Ast A, | Buntru A, | Schindler F, | Hasenkopf R, | Schulz A, | Brusendorf L, | Klockmeier K, | Grelle G, | McMahon B, | Niederlechner H, | Jansen I, | Diez L, | Edel J, | Boeddrich A, | Franklin SA, | Baldo B, | Schnoegl S, | Kunz S, | Purfürst B, | Gaertner A, | Kampinga HH, | Morton AJ, | Petersén Å, | Kirstein J, | Bates GP, | Wanker EE |

mHTT seeding activity: a marker of disease progression and neurotoxicity in models of Huntington's disease

Mol Cell

675-688

71

(5):

20180906

PMID:30193095

DOI: 10.1016/j.molcel.2018.07.032

PMCID:

Evers MM, | Miniarikova J, | Juhas S, | Vallès A, | Bohuslavova B, | Juhasova J, | Skalnikova HK, | Vodicka P, | Valekova I, | Brouwers C, | Blits B, | Lubelski J, | Kovarova H, | Ellederova Z, | van Deventer SJ, | Petry H, | Motlik J, | Konstantinova P |

AAV5-miHTT gene therapy demonstrates broad distribution and strong human mutant huntingtin lowering in a Huntington's disease minipig model

Mol Ther

2163-2177

26

(9):

20180905

PMID:30007561

DOI: 10.1016/j.ymthe.2018.06.021

PMCID: 6127509

Didiot MC, | Ferguson CM, | Ly S, | Coles AH, | Smith AO, | Bicknell AA, | Hall LM, | Sapp E, | Echeverria D, | Pai AA, | DiFiglia M, | Moore MJ, | Hayward LJ, | Aronin N, | Khvorova A |

Nuclear localization of Huntingtin mRNA is specific to cells of neuronal origin

Cell Rep

2553-2560

24

(10):

20180904

PMID:30184490

DOI: 10.1016/j.celrep.2018.07.106

PMCID:

Goodliffe JW, | Song H, | Rubakovic A, | Chang W, | Medalla M, | Weaver CM, | Luebke JI |

Differential changes to D1 and D2 medium spiny neurons in the 12-month-old Q175+/- mouse model of Huntington's disease

PLoS One

e0200626

13

(8):

20180817

PMID:30118496

DOI: 10.1371/journal.pone.0200626

PMCID: 6097649

Haraszti RA, | Miller R, | Didiot MC, | Biscans A, | Alterman JF, | Hassler MR, | Roux L, | Echeverria D, | Sapp E, | DiFiglia M, | Aronin N, | Khvorova A |

Optimized cholesterol-siRNA chemistry improves productive loading onto extracellular vesicles

Mol Ther

1973-1982

26

(8):

20180801

PMID:29937418

DOI: 10.1016/j.ymthe.2018.05.024

PMCID:

Wesson M, | Boileau NR, | Perlmutter JS, | Paulsen JS, | Barton SK, | McCormack MK, | Carlozzi NE |

Suicidal ideation assessment in individuals with premanifest and manifest Huntington disease

J Huntingtons Dis

239-249

7

(3):

20180731

PMID:30056431

DOI: 10.3233/JHD-180299

PMCID: 6108173

Pido-Lopez J, | Andre R, | Benjamin AC, | Ali N, | Farag S, | Tabrizi SJ, | Bates GP |

In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington's disease

Sci Rep

11447

8

(1):

20180730

PMID:30061661

DOI: 10.1038/s41598-018-29792-x

PMCID:

Mir A, | Alterman JF, | Hassler MR, | Debacker AJ, | Hudgens E, | Echeverria D, | Brodsky MH, | Khvorova A, | Watts JK, | Sontheimer EJ |

Heavily and fully modified RNAs guide efficient SpyCas9-mediated genome editing

Nat Commun

2641

9

(1):

20180706

PMID:29980686

DOI: 10.1038/s41467-018-05073-z

PMCID:

Ko J, | Isas JM, | Sabbaugh A, | Yoo JH, | Pandey NK, | Chongtham A, | Ladinsky M, | Wu WL, | Rohweder H, | Weiss A, | Macdonald D, | Munoz-Sanjuan I, | Langen R, | Patterson PH, | Khoshnan A |

Identification of distinct conformations associated with monomers and fibril assemblies of mutant huntingtin

Hum Mol Genet

2330–2343

27

(13):

20180701

PMID:29912367

DOI: 10.1093/hmg/ddy141

PMCID:

Verhaeghe J, | Bertoglio D, | Kosten L, | Thomae D, | Verhoye M, | Van Der Linden A, | Wyffels L, | Stroobants S, | Wityak J, | Dominguez C, | Mrzljak L, | Staelens S |

Noninvasive relative quantification of [(11)C]ABP688 PET imaging in mice versus an input function measured over an arteriovenous shunt

Front Neurol

9

(516):

20180629

PMID:30013509

DOI: 10.3389/fneur.2018.00516

PMCID: 6036254

Reif A, | Chiki A, | Ricci J, | Lashuel HA |

Generation of native, untagged Huntingtin Exon1 monomer and fibrils using a SUMO fusion strategy

J Vis Exp

136

():

20180627

PMID:30010666

DOI: 10.3791/57506

PMCID:

Biscans A, | Haraszti RA, | Echeverria D, | Miller R, | Didiot MC, | Nikan M, | Roux L, | Aronin N, | Khvorova A |

Hydrophobicity of lipid-conjugated siRNAs predicts productive loading to small extracellular vesicles

Mol Ther

1520-1528

26

(6):

20180606

PMID:29699940

DOI: 10.1016/j.ymthe.2018.03.019

PMCID:

Shin B, | Jung R, | Oh H, | Owens GE, | Lee H, | Kwak S, | Lee R, | Cotman SL, | Lee JM, | MacDonald ME, | Song JJ, | Vijayvargia R, | Seong IS |

Novel DNA aptamers that bind to mutant Huntingtin and modify its activity

Mol Ther Nucleic Acids

416-428

11

():

20180601

PMID:29858077

DOI: 10.1016/j.omtn.2018.03.008

PMCID: 5992459

Covey DP, | Dantrassy HM, | Yohn SE, | Castro A, | Conn PJ, | Mateo Y, | Cheer JF |

Inhibition of endocannabinoid degradation rectifies motivational and dopaminergic deficits in the Q175 mouse model of Huntington's disease

Neuropsychopharmacology

2056–2063

43

():

20180601

PMID:29925886

DOI: 10.1038/s41386-018-0107-8

PMCID: 6098121

Suelves N, | Miguez A, | López-Benito S, | Barriga GG, | Giralt A, | Alvarez-Periel E, | Arévalo JC, | Alberch J, | Ginés S, | Brito V |

Early downregulation of p75(NTR) by genetic and pharmacological approaches delays the onset of motor deficits and striatal dysfunction in Huntington's disease mice

Mol Neurobiol

():

20180528

PMID:29804232

DOI: 10.1007/s12035-018-1126-5

PMCID:

Martin DDO, | Kay C, | Collins JA, | Nguyen YT, | Slama RA, | Hayden MR |

A human huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington disease

Sci Rep

8096

9

(1):

20180525

PMID:29802276

DOI: 10.1038/s41598-018-25903-w

PMCID:

Bertoglio D, | Kosten L, | Verhaeghe J, | Thomae D, | Wyffels L, | Stroobants S, | Wityak J, | Dominguez C, | Mrzljak L, | Staelens S |

Longitudinal characterization of mGluR5 using (11)C-ABP688 PET imaging in the Q175 mouse model of Huntington's disease

J Nucl Med

1723-1727

59

(11):

20180524

PMID:29794227

DOI: 10.2967/jnumed.118.210658

PMCID:

McClory H, | Wang X, | Sapp E, | Gatune LW, | Iuliano M, | Wu CY, | Nathwani G, | Kegel-Gleason KB, | DiFiglia M, | Li X |

The COOH-terminal domain of huntingtin interacts with RhoGEF kalirin and modulates cell survival

Sci Rep

8

(1):

20180522

PMID:29789657

DOI: 10.1038/s41598-018-26255-1

PMCID:

Schuldenzucker V, | Schubert R, | Muratori LM, | Freisfeld F, | Rieke L, | Matheis T, | Schramke S, | Motlik J, | Kemper N, | Radespiel U, | Reilmann R |

Behavioral assessment of stress compensation in minipigs transgenic for the Huntington gene using cortisol levels: a proof-of-concept study

J Huntingtons Dis

151-161

7

(2):

20180519

PMID:29843247

DOI: 10.3233/JHD-180285

PMCID:

Gregory S, | Long JD, | Klöppel S, | Razi A, | Scheller E, | Minkova L, | Johnson EB, | Durr A, | Roos RAC, | Leavitt BR, | Mills JA, | Stout JC, | Scahill RI, | Tabrizi SJ, | Rees G, | Track-On investigators |

Testing a longitudinal compensation model in premanifest Huntington's disease

Brain

():

20180518

PMID:29788038

DOI: 10.1093/brain/awy122

PMCID:

Chao MJ, | Kim KH, | Shin JW, | Lucente D, | Wheeler VC, | Li H, | Roach JC, | Hood L, | Wexler NS, | Jardim LB, | Holmans P, | Jones L, | Orth M, | Kwak S, | MacDonald ME, | Gusella JF, | Lee JM |

Population-specific genetic modification of Huntington's disease in Venezuela

PLoS Genet

e1007274

14

(5):

20180511

PMID:29750799

DOI: 10.1371/journal.pgen.1007274

PMCID:

Mestre TA, | Busse M, | Davis AM, | Quinn L, | Rodrigues FB, | Burgunder JM, | Carlozzi NE, | Walker F, | Ho AK, | Sampaio C, | Goetz CG, | Cubo E, | Martinez-Martin P, | Stebbins GT, | Members of the MDS Committee on Rating Scales Development |

Rating scales and performance-based measures for assessment of functional ability in Huntington's disease: critique and recommendations

Mov Disord Clin Pract

361-372

5

(4):

20180509

PMID:30363510

DOI: 10.1002/mdc3.12617

PMCID: 6174516

Castro E, | Polosecki P, | Rish I, | Pustina D, | Warner JH, | Wood A, | Sampaio C, | Cecchi GA |

Baseline multimodal information predicts future motor impairment in premanifest Huntington's disease

Neuroimage Clin

443-453

19

():

20180509

PMID:29984153

DOI: 10.1016/j.nicl.2018.05.008

PMCID: 6029560

Gregory S, | Crawford H, | Seunarine K, | Leavitt B, | Durr A, | Roos RAC, | Scahill RI, | Tabrizi SJ, | Rees G, | Langbehn D, | Orth M |

Natural biological variation of white matter microstructure is accentuated in Huntington's disease

Hum Brain Mapp

():

20180422

PMID:29682858

DOI: 10.1002/hbm.24191

PMCID:

Beckmann H, | Bohlen S, | Saft C, | Hoffmann R, | Gerss J, | Muratori L, | Ringelstein EB, | Landwehrmeyer GB, | Reilmann R |

Objective assessment of gait and posture in premanifest and manifest Huntington disease - A multi-center study

Gait Posture

451-457

62

():

20180417

PMID:29660633

DOI: 10.1016/j.gaitpost.2018.03.039

PMCID:

Reiner A, | Deng YP |

Disrupted striatal neuron inputs and outputs in Huntington's disease

CNS Neurosci Ther

250-280

24

(4):

20180402

PMID:29582587

DOI: 10.1111/cns.12844

PMCID: 5875736

Carlozzi NE, | Hahn EA, | Goodnight SM, | Kratz AL, | Paulsen JS, | Stout JC, | Frank S, | Miner JA, | Cella D, | Gershon RC, | Schilling SG, | Ready RE |

Patient-reported outcome measures in Huntington disease: Quality of life in neurological disorders (Neuro-QoL) social functioning measures

Psychol Assess

450-458

30

(4):

20180402

PMID:28557480

DOI: 10.1037/pas0000479

PMCID: 5708162

Wijeratne PA, | Young AL, | Oxtoby NP, | Marinescu RV, | Firth NC, | Johnson EB, | Mohan A, | Sampaio C, | Scahill RI, | Tabrizi SJ, | Alexander DC |

An image-based model of brain volume biomarker changes in Huntington's disease

Ann Clin Transl Neurol

570-582

5

(5):

20180402

PMID:29761120

DOI: 10.1002/acn3.558

PMCID: 5945962

Lai JS, | Goodnight S, | Downing NR, | Ready RE, | Paulsen JS, | Kratz AL, | Stout JC, | McCormack MK, | Cella D, | Ross C, | Russell J, | Carlozzi NE |

Evaluating cognition in individuals with Huntington disease: Neuro-QoL cognitive functioning measures

Qual Life Res

811-822

27

(3):

20180330

PMID:29222609

DOI: 10.1007/s11136-017-1755-6

PMCID: 5845825

Baake V, | Coppen EM, | van Duijn E, | Dumas EM, | van den Bogaard SJA, | Scahill RI, | Johnson H, | Leavitt B, | Durr A, | Tabrizi SJ, | Craufurd D, | Roos RAC, | Track-HD investigators |

Apathy and atrophy of subcortical brain structures in Huntington's disease: A two-year follow-up study

Neuroimage Clin

66-70

19

():

20180327

PMID:30035003

DOI: 10.1016/j.nicl.2018.03.033

PMCID: 6051315

Ament SA, | Pearl JR, | Cantle JP, | Bragg RM, | Skene PJ, | Coffey SR, | Bergey DE, | Wheeler VC, | MacDonald ME, | Baliga NS, | Rosinski J, | Hood LE, | Carroll JB, | Price ND |

Transcriptional regulatory networks underlying gene expression changes in Huntington's disease

Mol Syst Biol

e7435

14

(3):

20180326

PMID:29581148

DOI: 10.15252/msb.20167435

PMCID: 5868199

Mestre TA, | Carlozzi NE, | Ho AK, | Burgunder JM, | Walker F, | Davis AM, | Busse M, | Quinn L, | Rodrigues FB, | Sampaio C, | Goetz CG, | Cubo E, | Martinez-Martin P, | Stebbins GT, | Members of the MDS Committee on Rating Scales Development|

Quality of life in Huntington's disease: Critique and recommendations for measures assessing patient health-related quality of life and caregiver quality of life

Mov Disord

742-749

33

(5):

20180323

PMID:29570848

DOI: 10.1002/mds.27317

PMCID:

Bresciani A, | Spiezia MC, | Boggio R, | Cariulo C, | Nordheim A, | Altobelli R, | Kuhlbrodt K, | Dominguez C, | Munoz-Sanjuan I, | Wityak J, | Fodale V, | Marchionini DM, | Weiss A |

Quantifying autophagy using novel LC3B and p62 TR-FRET assays

PLoS One

e0194423

13

(3):

20180319

PMID:29554128

DOI: 10.1371/journal.pone.0194423

PMCID: 5858923

Ouk K, | Aungier J, | Cuesta M, | Morton AJ |

Chronic paroxetine treatment prevents disruption of methamphetamine-sensitive circadian oscillator in a transgenic mouse model of Huntington's disease

Neuropharmacology

337-350

131

():

20180315

PMID:29274752

DOI: 10.1016/j.neuropharm.2017.12.033

PMCID:

Minkova L, | Gregory S, | Scahill RI, | Abdulkadir A, | Kaller CP, | Peter J, | Long JD, | Stout JC, | Reilmann R, | Roos RA, | Durr A, | Leavitt BR, | Tabrizi SJ, | Klöppel S, | TRACK-HD Investigators |

Cross-sectional and longitudinal voxel-based grey matter asymmetries in Huntington's disease

Neuroimage Clin

312-324

17

():

20180313

PMID:29527479

DOI: 10.1016/j.nicl.2017.10.023

PMCID: 5842644

Espinoza FA, | Turner JA, | Vergara VM, | Miller RL, | Mennigen E, | Liu J, | Misiura MB, | Ciarochi J, | Johnson HJ, | Long JD, | Bockholt HJ, | Magnota VA, | Paulsen JS, | Calhoun VD |

Whole-brain connectivity in a large study of Huntington's disease gene mutation carriers and healthy controls

Brain Connect

():

20180308

PMID:29291624

DOI: 10.1089/brain.2017.0538

PMCID:

Lee CYD, | Daggett A, | Gu X, | Jiang LL, | Langfelder P, | Li X, | Wang N, | Zhao Y, | Park CS, | Cooper Y, | Ferando I, | Mody I, | Coppola G, | Xu H, | Yang XW |

Elevated TREM2 gene dosage reprograms microglia responsivity and ameliorates pathological phenotypes in Alzheimer's disease models

Neuron

1032-1048

97

(5):

20180307

PMID:29518357

DOI: 10.1016/j.neuron.2018.02.002

PMCID:

Guo Q, | Bin Huang, | Cheng J, | Seefelder M, | Engler T, | Pfeifer G, | Oeckl P, | Otto M, | Moser F, | Maurer M, | Pautsch A, | Baumeister W, | Fernández-Busnadiego R, | Kochanek S |

The cryo-electron microscopy structure of huntingtin

Nature

117-120

555

(7694):

20180301

PMID:29466333

DOI: 10.1038/nature25502

PMCID:

Odish OFF, | Reijntjes RHAM, | van den Bogaard SJA, | Roos RAC, | Leemans A |

Progressive microstructural changes of the occipital cortex in Huntington's disease

Brain Imaging Behav

():

20180228

PMID:29492750

DOI: 10.1007/s11682-018-9849-5

PMCID:

Kielar C, | Morton AJ |

Early neurodegeneration in R6/2 mice carrying the Huntington's disease mutation with a super-expanded CAG repeat, despite normal lifespan

J Huntingtons Dis

61-76

7

(1):

20180227

PMID:29480204

DOI: 10.3233/JHD-170265

PMCID:

Kovalenko M, | Milnerwood A, | Giordano J, | St Claire J, | Guide JR, | Stromberg M, | Gillis T, | Sapp E, | DiFiglia M, | MacDonald ME, | Carroll JB, | Lee JM, | Tappan S, | Raymond L, | Wheeler VC |

HttQ111/+ Huntington's disease knock-in mice exhibit brain region-specific morphological changes and synaptic dysfunction

J Huntingtons Dis

17-33

7

(1):

20180224

PMID:29480209

DOI: 10.3233/JHD-170282

PMCID:

Pfister EL, DiNardo N, Mondo E, Borel F, Conroy F, Fraser C, | Gernoux G, | Han X, | Hu D, | Johnson E, | Kennington L, | Liu P, | Reid SJ, | Sapp E, | Vodicka P, | Kuchel T, | Morton AJ, | Howland D, | Moser R, | Sena-Esteves M, | Gao G, | Mueller C, | DiFiglia M, | Aronin N |

Artificial miRNAs reduce Human Mutant Huntingtin throughout the striatum in a transgenic sheep model of Huntington's disease

Hum Gene Ther

():

20180223

PMID:29207890

DOI: 10.1089/hum.2017.199

PMCID:

Veldman MB, | Yang XW |

Molecular insights into cortico-striatal miscommunications in Huntington's disease

Curr Opin Neurobiol

79-89

48

():

20180222

PMID:29125980

DOI: 10.1016/j.conb.2017.10.019

PMCID: 5825262

Rebec GV |

Corticostriatal network dysfunction in huntington's disease: Deficits in neural processing, glutamate transport, and ascorbate release

CNS Neurosci Ther

281-291

24

(4):

20180221

PMID:29464896

DOI: 10.1111/cns.12828

PMCID:

Sampaio C, | Levey J, | Klitzman R |

Predictive testing and clinical trials in Huntington's disease: An ethical analysis

Mov Disord

243-247

33

(2):

20180220

PMID:29205501

DOI: 10.1002/mds.27247

PMCID: 5854324

Mestre TA, | Bachoud-Lévi AC, | Marinus J, | Stout JC, | Paulsen JS, | Como P, | Duff K, | Sampaio C, | Goetz CG, | Cubo E, | Stebbins GT, | Martinez-Martin P, | Members of the MDS Committee on Rating Scales Development |

Rating scales for cognition in Huntington's disease: Critique and recommendations

Mov Disord

187-195

33

(2):

20180219

PMID:29278291

DOI: 10.1002/mds.27227

PMCID:

Hassler MR, | Turanov AA, | Alterman JF, | Haraszti RA, | Coles AH, | Osborn MF, | Echeverria D, | Nikan M, | Salomon WE, | Roux L, | Godinho BMDC, | Davis SM, | Morrissey DV, | Zamore PD, | Karumanchi SA, | Moore MJ, | Aronin N, | Khvorova A |

Comparison of partially and fully chemically-modified siRNA in conjugate-mediated delivery in vivo

Nucleic Acids Res

1-12

():

20180208

PMID:29432571

DOI: 10.1093/nar/gky037

PMCID:

Harrison DJ | Roberton VH | Vinh NN | Brooks SP | Dunnett SB | Rosser AE |

The effect of tissue preparation and donor age on striatal graft morphology in the mouse

Cell Transplant

230-244

27

(2):

20180202

PMID:29637815

DOI: 10.1177/0963689717744788

PMCID: 5898691

Arnerić SP, | Kern VD, | Stephenson DT |

Regulatory-accepted drug development tools are needed to accelerate innovative CNS disease treatments

Biochem Pharmacol

291-306

151

():

20180130

PMID:29410157

DOI: 10.1016/j.bcp.2018.01.043

PMCID:

Ruzo A, | Croft GF, | Metzger JJ, | Galgoczi S, | Gerber LJ, | Pellegrini C, | Wang H Jr, | Fenner M, | Tse S, | Marks A, | Nchako C, | Brivanlou AH |

Chromosomal instability during neurogenesis in Huntington's disease

Development

dev156844

145

(2):

20180129

PMID:29378824

DOI: 10.1242/dev.156844

PMCID:

Johnson EB, | Byrne LM, | Gregory S, | Rodrigues FB, | Blennow K, | Durr A, | Leavitt BR, | Roos RA, | Zetterberg H, | Tabrizi SJ, | Scahill RI, | Wild EJ, | TRACK-HD Study Group |

Neurofilament light protein in blood predicts regional atrophy in Huntington disease

Neurology

():

20180124

PMID:29367444

DOI: 10.1212/WNL.0000000000005005

PMCID:

Casula EP, | Mayer IMS, | Desikan M, | Tabrizi SJ, | Rothwell JC, | Orth M |

Motor cortex synchronization influences the rhythm of motor performance in premanifest Huntington's disease

Mov Disord

440-448

33

(3):

20180122

PMID:29356133

DOI: 10.1002/mds.27285

PMCID:

Sepers MD, | Smith-Dijak A, | LeDue J, | Kolodziejczyk K, | Mackie K, | Raymond LA |

Endocannabinoid-specific impairment in synaptic plasticity in striatum of Huntington's disease mouse model

J Neurosci

544-554

38

(3):

20180117

PMID:29192125

DOI: 10.1523/JNEUROSCI.1739-17.2017

PMCID:

Langfelder P, | Gao F, | Wang N, | Howland D, | Kwak S, | Vogt TF, | Aaronson JS, | Rosinski J, | Coppola G, | Horvath S, | Yang XW |

MicroRNA signatures of endogenous Huntingtin CAG repeat expansion in mice

PLoS One

e0190550

13

(1):

20180111

PMID:29324753

DOI: 10.1371/journal.pone.0190550

PMCID:

Carlozzi NE, | Hahn EA, | Frank SA, | Perlmutter JS, | Downing ND, | McCormack MK, | Barton S, | Nance MA, | Schilling SG, | HDQLIFE Site Investigators and Coordinators |

A new measure for end of life planning, preparation, and preferences in Huntington disease: HDQLIFE end of life planning

J Neurol

98-107

265

(1):

20180110

PMID:29143208

DOI: 10.1007/s00415-017-8677-7

PMCID: 5762414

Sbodio JI, | Snyder SH, | Paul BD |

Golgi stress response reprograms cysteine metabolism to confer cytoprotection in Huntington's disease

Proc Natl Acad Sci USA

():

20180109

PMID:29317536

DOI: 10.1073/pnas.1717877115

PMCID:

Conforti P, | Besusso D, | Bocchi VD, | Faedo A, | Cesana E, | Rossetti G, | Ranzani V, | Svendsen CN, | Thompson LM, | Toselli M, | Biella G, | Pagani M, | Cattaneo E |

Faulty neuronal determination and cell polarization are reverted by modulating HD early phenotypes

Proc Natl Acad Sci USA

E762-E771

115

(4):

20180108

PMID:29311338

DOI: 10.1073/pnas.1715865115

PMCID:

Wang HB, | Loh DH, | Whittaker DS, | Cutler T, | Howland D, | Colwell CS |

Time-restricted feeding improves circadian dysfunction as well as motor symptoms in the Q175 mouse model of Huntington's disease

eNeuro

5

(1):

20180103

PMID:29302618

DOI: 10.1523/ENEURO.0431-17.2017

PMCID: 5752678

2017

Al-Ramahi I, | Panapakkam Giridharan SS, | Chen YC, | Patnaik S, | Safren N, | Hasegawa J, | de Haro M, | Wagner Gee AK, | Titus SA, | Jeong H, | Clarke J, | Krainc D, | Zheng W, | Irvine RF, | Barmada S, | Ferrer M, | Southall N, | Weisman LS, | Botas J, | Marugan JJ |

Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein

elife

e29123

6

():

20171226

PMID:29256861

DOI: 10.7554/eLife.29123

PMCID: 5743427

Hensman Moss DJ, | Robertson N, | Farmer R, | Scahill RI, | Haider S, | Tessari MA, | Flynn G, | Fischer DF, | Wild EJ, | Macdonald D, | Tabrizi SJ |

Quantification of huntingtin protein species in Huntington's disease patient leukocytes using optimised electrochemiluminescence immunoassays

PLoS One

e0189891

12

(12):

20171222

PMID:29272284

DOI: 10.1371/journal.pone.0189891

PMCID:

Fodale V, | Boggio R, | Daldin M, | Cariulo C, | Spiezia MC, | Byrne LM, | Leavitt BR, | Wild EJ, | Macdonald D, | Weiss A, | Bresciani A |

Validation of ultrasensitive mutant Huntingtin detection in human cerebrospinal fluid by single molecule counting immunoassay

J Huntingtons Dis

349-361

6

(4):

20171212

PMID:29125493

DOI: 10.3233/JHD-170269

PMCID:

Schobel SA, | Palermo G, | Auinger P, | Long JD, | Ma S, | Khwaja OS, | Trundell D, | Cudkowicz M, | Hersch S, | Sampaio C, | Dorsey ER, | Leavitt BR, | Kieburtz KD, | Sevigny JJ, | Langbehn DR, | Tabrizi SJ, | TRACK-HD, | COHORT, | CARE-HD, | and 2CARE Huntington Study Group Investigators |

Motor, cognitive, and functional declines contribute to a single progressive factor in early HD

Neurology

2495-2502

89

(24):

20171212

PMID:29142089

DOI: 10.1212/WNL.0000000000004743

PMCID: 5729794

Dickey AS, | Sanchez DN, | Arreola M, | Sampat KR, | Fan W, | Arbez N, | Akimov S, | Van Kanegan MJ, | Ohnishi K, | Gilmore-Hall SK, | Flores AL, | Nguyen JM, | Lomas N, | Hsu CL, | Lo DC, | Ross CA, | Masliah E, | Evans RM, | La Spada AR |

PPARδ activation by bexarotene promotes neuroprotection by restoring bioenergetic and quality control homeostasis

Sci Transl Med

eaal2332

9

(419):

20171206

PMID:29212711

DOI: 10.1126/scitranslmed.aal2332

PMCID: 5748332

Arbez N, | Ratovitski T, | Roby E, | Chighladze E, | Stewart JC, | Ren M, | Wang X, | Lavery DJ, | Ross CA|

Post-translational modifications clustering within proteolytic domains decrease mutant huntingtin toxicity

J Biol Chem

19238-19249

292

(47):

20171124

PMID:28972180

DOI: 10.1074/jbc.M117.782300

PMCID:

Friedman A, | Homma D, | Bloem B, | Gibb LG, | Amemori KI, | Hu D, | Delcasso S, | Truong TF, | Yang J, | Hood AS, | Mikofalvy KA, | Beck DW, | Nguyen N, | Nelson ED, | Toro Arana SE, | Vorder Bruegge RH, | Goosens KA, | Graybiel AM |

Chronic stress alters striosome-circuit dynamics, leading to aberrant decision-making

Cell

1191-1205

171

(5):

20171116

PMID:29149606

DOI: 10.1016/j.cell.2017.10.017

PMCID: 5734095

Chao MJ, | Gillis T, | Atwal RS, | Mysore JS, | Arjomand J, | Harold D, | Holmans P, | Jones L, | Orth M, | Myers RH, | Kwak S, | Wheeler VC, | MacDonald ME, | Gusella JF, | Lee JM |

Haplotype-based stratification of Huntington's disease

Eur J Hum Genet

1202-1209

25

(11):

20171101

PMID:28832564

DOI: 10.1038/ejhg.2017.125

PMCID:

Brown KE, | Lohse KR, | Mayer IMS, | Strigaro G, | Desikan M, | Casula EP, | Meunier S, | Popa T, | Lamy JC, | Odish O, | Leavitt BR, | Durr A, | Roos RAC, | Tabrizi SJ, | Rothwell JC, | Boyd LA, | Orth M |

The reliability of commonly used electrophysiology measures

Brain Stimul

1102-1111

10

(6):

20171101

PMID:28807846

DOI: 10.1016/j.brs.2017.07.011

PMCID:

Wu D, | Faria AV, | Younes L, | Mori S, | Brown T, | Johnson H, | Paulsen JS, | Ross CA, | Miller MI, | PREDICT-HD Investigators |Coordinators of the Huntington Study Group |

Mapping the order and pattern of brain structural MRI changes using change-point analysis in premanifest Huntington's disease

Hum Brain Mapp

5035-5050

38

(10):

20171031

PMID:28657159

DOI: 10.1002/hbm.23713

PMCID:

Arnerić SP, | Cedarbaum JM, | Khozin S, | Papapetropoulos S, | Hill DL, | Ropacki M, | Rhodes J, | Dacks PA, | Hudson LD, | Gordon MF, | Kern VD, | Romero K, | Vradenburg G, | Au R, | Karlin DR, | Facheris MF, | Fitzer-Attas CJ, | Vitolo OV, | Wang J, | Miller BM, | Kaye JA |

Biometric monitoring devices for assessing end points in clinical trials: developing an ecosystem

Nat Rev Drug Discov

16

(10):

20171031

PMID:28935908

DOI: 10.1038/nrd.2017.153

PMCID:

Wild EJ, | Tabrizi SJ |

Therapies targeting DNA and RNA in Huntington's disease

Lancet Neurol

837-847

16

(10):

20171031

PMID:28920889

DOI: 10.1016/S1474-4422(17)30280-6

PMCID:

Whittaker DS, | Wang HB, | Loh DH, | Cachope R, | Colwell CS |

Possible use of a H3R antagonist for the management of nonmotor symptoms in the Q175 mouse model of Huntington's disease

Pharmacol Res Perspect

e00344

5

(5):

20171030

PMID:28971617

DOI: 10.1002/prp2.344

PMCID: 5625154

Bondulich MK, | Jolinon N, | Osborne GF, | Smith EJ, | Rattray I, | Neueder A, | Sathasivam K, | Ahmed M, | Ali N, | Benjamin AC, | Chang X, | Dick JRT, | Ellis M, | Franklin SA, | Goodwin D, | Inuabasi L, | Lazell H, | Lehar A, | Richard-Londt A, | Rosinski J, | Smith DL, | Wood T, | Tabrizi SJ, | Brandner S, | Greensmith L, | Howland D, | Munoz-Sanjuan I, | Lee SJ, | Bates GP |

Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice

Sci Rep

14275

7

(1):

20171027

PMID:29079832

DOI: 10.1038/s41598-017-14290-3

PMCID:

Warner JB 4th, | Ruff KM, | Tan PS, | Lemke EA, | Pappu RV, | Lashuel HA |

Monomeric Huntingtin Exon 1 has similar overall structural features for wild-type and pathological polyglutamine lengths

J Am Chem Soc

14456-14469

139

(41):

20171018

PMID:28937758

DOI: 10.1021/jacs.7b06659

PMCID:

Schuldenzucker V, | Schubert R, | Muratori LM, | Freisfeld F, | Rieke L, | Matheis T, | Schramke S, | Motlik J, | Kemper N, | Radespiel U, | Reilmann R |

Behavioral testing of minipigs transgenic for the Huntington gene - A three-year observational study

PLoS One

e0185970

12

(10):

20171009

PMID:29016656

DOI: 10.1371/journal.pone.0185970

PMCID: 5633197

Bresciani A, | Missineo A, | Gallo M, | Cerretani M, | Fezzardi P, | Tomei L, | Cicero DO, | Altamura S, | Santoprete A, | Ingenito R, | Bianchi E, | Pacifici R, | Dominguez C, | Munoz-Sanjuan I, | Harper S, | Toledo-Sherman L, | Park LC |

Nuclear factor (erythroid-derived 2)-like 2 (NRF2) drug discovery: Biochemical toolbox to develop NRF2 activators by reversible binding of Kelch-like ECH-associated protein 1 (KEAP1)

Arch Biochem Biophys

31-41

631

():

20171001

PMID:28801166

DOI: 10.1016/j.abb.2017.08.003

PMCID:

Lee JM, | Chao MJ, | Harold D, | Abu Elneel K, | Gillis T, | Holmans P, | Jones L, | Orth M, | Myers RH, | Kwak S, | Wheeler VC, | MacDonald ME, | Gusella JF |

A modifier of Huntington's disease onset at the MLH1 locus

Hum Mol Genet

3859-3867

26

(19):

20171001

PMID:28934397

DOI: 10.1093/hmg/ddx286

PMCID:

Long JD, | Mills JA, | Leavitt BR, | Durr A, | Roos RA, | Stout JC, | Reilmann R, | Landwehrmeyer B, | Gregory S, | Scahill RI, | Langbehn DR, | Tabrizi SJ, | Track-HD and Track-On Investigators |

Survival end points for Huntington disease trials prior to a motor diagnosis

JAMA Neurol

():

20170918

PMID:28975278

DOI: 10.1001/jamaneurol.2017.2107

PMCID:

Frost C, | Mulick A, | Scahill RI, | Owen G, | Aylward E, | Leavitt BR, | Durr A, | Roos RAC, | Borowsky B, | Stout JC, | Reilmann R, | Langbehn DR, | Tabrizi SJ, | Sampaio C, | TRACK-HD Investigators |

Design optimization for clinical trials in early-stage manifest Huntington's disease

Mov Disord

1610-1619

32

(11):

20170914

PMID:28906031

DOI: 10.1002/mds.27122

PMCID:

Niccolini F, | Pagano G, | Fusar-Poli P, | Wood A, | Mrzljak L, | Sampaio C, | Politis M |

Striatal molecular alterations in HD gene carriers: a systematic review and meta-analysis of PET studies

J Neurol Neurosurg Psychiatry

():

20170909

PMID:28889093

DOI: 10.1136/jnnp-2017-316633

PMCID:

Westervelt HJ, | Bernier RA, | Faust M, | Gover M, | Bockholt HJ, | Zschiegner R, | Long JD, | Paulsen JS, | PREDICT-HD Investigators, | Coordinators of the Huntington Study Group |

Data quality assurance and control in cognitive research: Lessons learned from the PREDICT-HD study

Int J Methods Psychiatr Res

1-9

26

(3):

20170901

PMID:28211597

DOI: 10.1002/mpr.1534

PMCID:

Hensman Moss DJ, | Pardiñas AF, | Langbehn D, | Lo K, | Leavitt BR, | Roos R, | Durr A, | Mead S, | TRACK-HD investigators, | REGISTRY investigators, | Holmans P, | Jones L, | Tabrizi SJ |

Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

Lancet Neurol

701-711

16

(9):

20170901

PMID:28642124

DOI: 10.1016/S1474-4422(17)30161-8

PMCID:

Brady ST, | Morfini GA |

Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases

Neurobiol Dis

273-282

105

():

20170901

PMID:28411118

DOI: 10.1016/j.nbd.2017.04.010

PMCID: 5522763

Schain M, | Fazio P, | Mrzljak L, | Amini N, | Al-Tawil N, | Fitzer-Attas C, | Bronzova J, | Landwehrmeyer B, | Sampaio C, | Halldin C, | Varrone A |

Revisiting the Logan plot to account for non-negligible blood volume in brain tissue

EJNMMI Res

7

(66):

20170818

PMID:28822101

DOI: 10.1186/s13550-017-0314-z

PMCID: 5561763

Ratovitski T, | O'Meally RN, | Jiang M, | Chaerkady R, | Chighladze E, | Stewart JC, | Wang X, | Arbez N, | Roby E, | Alexandris A, | Duan W, | Vijayvargia R, | Seong IS, | Lavery DJ, | Cole RN, | Ross CA |

Post-Translational Modifications (PTMs), identified on endogenous Huntingtin, cluster within proteolytic domains between HEAT repeats

J Proteome Res

2692-2708

16

(8):

20170804

PMID:28653853

DOI: 10.1021/acs.jproteome.6b00991

PMCID: 5560079

Zheng P, | Kozloski J |

Striatal network models of Huntington's disease dysfunction phenotypes

Front Comput Neurosci

11

(70):

20170727

PMID:28798680

DOI: 10.3389/fncom.2017.00070

PMCID: 5529396

Ghosh S, | Sun Z, | Li Y, | Cheng Y, | Mohan A, | Sampaio C, | Hu J |

An exploration of latent structure in observational Huntington's disease studies

AMIA Jt Summits Transl Sci Proc

92-102

2017

():

20170726

PMID:28815114

DOI:

PMCID: 5543350

Daldin M, | Fodale V, | Cariulo C, | Azzollini L, | Verani M, | Martufi P, | Spiezia MC, | Deguire SM, | Cherubini M, | Macdonald D, | Weiss A, | Bresciani A, | Vonsattel JG, | Petricca L, | Marsh JL, | Gines S, | Santimone I, | Marano M, | Lashuel HA. | Squitieri F, | Caricasole A |

Polyglutamine expansion affects huntingtin conformation in multiple Huntington's disease models

Sci Reports

1-13

7

(5070):

20170711

PMID:28698602

DOI: 10.1038/s41598-017-05336-7

PMCID: 5505970

Kang H, | Vázquez FX, | Zhang L, | Das P, | Toledo-Sherman L, | Luan B, | Levitt M, | Zhou R |

Emerging β-sheet rich conformations in supercompact Huntingtin Exon-1 mutant structures

J Am Chem Soc

8820-8827

139

(26):

20170705

PMID:28609090

DOI: 10.1021/jacs.7b00838

PMCID:

Khakh BS, | Beaumont V, | Cachope R, | Munoz-Sanjuan I, | Goldman SA, | Grantyn R |

Unravelling and exploiting astrocyte dysfunction in Huntington's disease

Trends Neurosci

422-437

40

(7):

20170701

PMID:28578789

DOI: 10.1016/j.tins.2017.05.002

PMCID:

Dominguez D JF, | Poudel G, | Stout JC, | Gray M, | Chua P, | Borowsky B, | Egan GF, | Georgiou-Karistianis N |

Longitudinal changes in the fronto-striatal network are associated with executive dysfunction and behavioral dysregulation in Huntington's disease : 30 months IMAGE-HD data

Cortex

139-149

92

():

20170701

PMID:28482181

DOI: 10.1016/j.cortex.2017.04.001

PMCID:

Nikan M, | Osborn MF, | Coles AH, | Biscans A, | Godinho BMDC, | Haraszti RA, | Sapp E, | Echeverria D, | DiFiglia M, | Aronin N, | Khvorova A |

Synthesis and evaluation of parenchymal retention and efficacy of a metabolically stable O-Phosphocholine-N-docosahexaenoyl-l-serine siRNA conjugate in mouse brain

Bioconjug Chem

1758-1766

28

(6):

20170621

PMID:28462988

DOI: 10.1021/acs.bioconjchem.7b00226

PMCID:

Teka WW, | Hamade KC, | Barnett WH, | Kim T, | Markin SN, | Rybak IA, | Molkov YI |

From the motor cortex to the movement and back again

PLoS One

e0179288

12

(6):

20170620

PMID:28632736

DOI: 10.1371/journal.pone.0179288

PMCID: 5478113

Byrne LM, | Rodrigues FB, | Blennow K, | Durr A, | Leavitt BR, | Roos RAC, | Scahill RI, | Tabrizi SJ, | Zetterberg H, | Langbehn D, | Wild EJ |

Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis

Lancet Neurol

S1474-4422

17

(30124-2):

20170607

PMID:28601473

DOI: 10.1016/S1474-4422(17)30124-2

PMCID:

Quinti L, | Dayalan Naidu S, | Träger U, | Chen X, | Kegel-Gleason K | Llères D, | Connolly C, | Chopra V, | Low C, | Moniot S, | Sapp E, | Tousley AR, | Vodicka P, | Van Kanegan MJ, | Kaltenbach LS, | Crawford LA, | Fuszard M, | Higgins M, | Miller JRC, | Farmer RE, | Potluri V, | Samajdar S, | Meisel L, | Zhang N, | Snyder A, | Stein R, | Hersch SM, | Ellerby LM, | Weerapana E, | Schwarzschild MA, | Steegborn C, | Leavitt BR, | Degterev A, | Tabrizi SJ, | Lo DC, | DiFiglia M, | Thompson LM, | Dinkova-Kostova AT, | Kazantsev AG |

KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patients

Proc Natl Acad Sci USA

E4676-E4685

114

(23):

20170606

PMID:28533375

DOI: 10.1073/pnas.1614943114

PMCID:

Cleary JD, | Ranum LP |

New developments in RAN translation: insights from multiple diseases

Curr Opin Genet Dev

125-134

44

():

20170601

PMID:28365506

DOI: 10.1016/j.gde.2017.03.006

PMCID:

McColgan P, | Razi A, | Gregory S, | Seunarine KK, | Durr A, | Roos ACR, | Leavitt BR, | Scahill RI, | Clark CA, | Langbehn DR, | Rees G, | Tabrizi SJ, | Track On-HD Investigators |

Structural and functional brain network correlates of depressive symptoms in premanifest Huntington's disease

Hum Brain Mapp

2819-2829

38

(6):

20170601

PMID:28294457

DOI: 10.1002/hbm.23527

PMCID: 5434856

Farina F, | Lambert E, | Commeau L, | Lejeune FX, | Roudier N, | Fonte C, | Parker JA, | Boddaert J, | Verny M, | Baulieu EE, | Neri C |

The stress response factor daf-16 FOXO is required for multiple compound families to prolong the function of neurons with Huntington's disease

Sci Rep

4014

7

(1):

20170601

PMID:28638078

DOI: 10.1038/s41598-017-04256-w

PMCID: 5479833

Fazio P, | Schain M, | Mrzljak L, | Amini N, | Nag S, | Al-Tawil N, | Fitzer-Attas CJ, | Bronzova J, | Landwehrmeyer B, | Sampaio C, | Halldin C, | Varrone A |

Patterns of age related changes for phosphodiesterase type-10A in comparison with dopamine D(2/3) receptors and sub-cortical volumes in the human basal ganglia: A PET study with (18)F-MNI-659 and (11)C-raclopride with correction for partial volume effect

Neuroimage

330-339

152

():

20170515

PMID:28254508

DOI: 10.1016/j.neuroimage.2017.02.047

PMCID:

Neueder A, | Landles C, | Ghosh R, | Howland D, | Myers RH, | Faull RLM, | Tabrizi SJ, | Bates GP |

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients

Sci Rep

1307

7

(1):

20170502

PMID:28465506

DOI: 10.1038/s41598-017-01510-z

PMCID: 5431000

Chiki A, | DeGuire SM, | Ruggeri FS, | Sanfelice D, | Ansaloni A, | Wang ZM, | Cendrowska U, | Burai R, | Vieweg S, | Pastore A, | Dietler G, | Lashuel HA |

Mutant exon1 Huntingtin aggregation is regulated by T3 phosphorylation-induced structural changes and crosstalk between T3 phosphorylation and acetylation at K6

Angew Chem Int Ed Engl

5202-5207

56

(19):

20170502

PMID:28334491

DOI: 10.1002/anie.201611750

PMCID:

HD iPSC Consortium

Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice

Nat Neurosci

648-660

20

(5):

20170501

PMID:28319609

DOI: 10.1038/nn.4532

PMCID: 5610046

Martín-Ibáñez R, | Guardia I, | Pardo M, | Herranz C, | Zietlow R, | Vinh NN, | Rosser A, | Canals JM |

Insights in spatio-temporal characterization of human fetal neural stem cells

Exp Neurol

20-35

291

():

20170501

PMID:28131724

DOI: 10.1016/j.expneurol.2017.01.011

PMCID:

Miller JRC, | Pfister EL, | Liu W, | Andre R, | Träger U, | Kennington LA, | Lo K, | Dijkstra S, | Macdonald D, | Ostroff G, | Aronin N, | Tabrizi SJ |

Allele-selective suppression of mutant Huntingtin in primary human blood cells

Sci Rep

46740

7

():

20170424

PMID:28436437

DOI: 10.1038/srep46740

PMCID: 5402279

Zhang L, | Kang H, | Vázquez FX, | Toledo-Sherman L, | Luan B. | Zhou R |

Molecular mechanism of stabilizing the helical structure of Huntingtin N17 in a micellar environment

J Phys Chem B

():

20170417

PMID:28413881

DOI: 10.1021/acs.jpcb.7b01476

PMCID:

Gregory S, | Long JD, | Klöppel S, | Razi A, | Scheller E, | Minkova L, | Papoutsi M, | Mills JA, | Durr A, | Leavitt BR, | Roos RAC, | Stout JC, | Scahill RI, | Langbehn DR, | Tabrizi SJ, | Rees G |

Operationalizing compensation over time in neurodegenerative disease

Brain

1158-1165

140

(4):

20170401

PMID:28334888

DOI: 10.1093/brain/awx022

PMCID: 5382953

Häggkvist J, | Tóth M, | Tari L, | Varnäs K, | Svedberg M, | Forsberg A, | Nag S, | Dominguez C, | Munoz-Sanjuan I, | Bard J, | Wityak J, | Varrone A, | Halldin C, | Mrzljak L |

Longitudinal small-animal PET imaging of the zQ175 mouse model of Huntington disease shows in vivo changes of molecular targets in the striatum and cerebral cortex

J Nucl Med

617-622

58

(4):

20170401

PMID:27856625

DOI: 10.2967/jnumed.116.180497

PMCID:

Wang JKT, | Langfelder P, | Horvath S, | Palazzolo MJ |

Exosomes and homeostatic synaptic plasticity are linked to each other and to Huntington's, Parkinson's, and other neurodegenerative diseases by database-enabled analyses of comprehensively curated datasets

Front Neurosci

11

(149):

20170331

PMID:28611571

DOI: 10.3389/fnins.2017.00149

PMCID: 5374209

Kim T, | Hamade KC, | Todorov D, | Barnett WH, | Capps RA, | Latash EM, | Markin SN, | Rybak IA, | Molkov YI |

Reward based motor adaptation mediated by basal ganglia

Front Comput Neurosci

11

(19):

20170331

PMID:28408878

DOI: 10.3389/fncom.2017.00019

PMCID: 5374212

Hensman Moss DJ, | Flower MD, | Lo KK, | Miller JR, | van Ommen GB, | 't Hoen PA, | Stone TC, | Guinee A, | Langbehn DR, | Jones L, | Plagnol V, | van Roon-Mom WM, | Holmans P, | Tabrizi SJ |

Huntington's disease blood and brain show a common gene expression pattern and share an immune signature with Alzheimer's disease

Sci Rep

44849

7

():

20170321

PMID:28322270

DOI: 10.1038/srep44849

PMCID: 5359597

Southwell AL, | Skotte NH, | Villanueva EB, | Østergaard ME, | Gu X, | Kordasiewicz HB, | Kay C, | Cheung D, | Xie Y, | Waltl S, | Dal Cengio L, | Findlay-Black H, | Doty CN, | Petoukhov E, | Iworima D, | Slama R, | Ooi J, | Pouladi MA, | Yang XW, | Swayze EE, | Seth PP, | Hayden MR |

A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles

Hum Mol Genet

1115-1132

26

(6):

20170315

PMID:28104789

DOI: 10.1093/hmg/ddx021

PMCID:

Ament SA, | Pearl JR, | Grindeland A, | St Claire J, | Earls JC, | Kovalenko M, | Gillis T, | Mysore J, | Gusella JF, | Lee JM, | Kwak S, | Howland D, | Lee MY, | Baxter D, | Scherler K, | Wang K, | Geman D, | Carroll JB, | MacDonald ME, | Carlson G, | Wheeler VC, | Price ND, | Hood LE |

High resolution time-course mapping of early transcriptomic, molecular and cellular phenotypes in Huntington's disease CAG knock-in mice across multiple genetic backgrounds

Hum Mol Genet

913-922

26

(5):

20170301

PMID:28334820

DOI: 10.1093/hmg/ddx006

PMCID:

Schultz JL,| Kamholz JA,| Moser DJ,| Feely SM,| Paulsen JS,| Nopoulos PC|

Substance abuse may hasten motor onset of Huntington disease: Evaluating the Enroll-HD database

Neurology

909-915

88

(9):

20170228

PMID:28148631

DOI: 10.1212/WNL.0000000000003661

PMCID:

Yu MS, | Tanese N |

Huntingtin is required for neural but not cardiac/pancreatic progenitor differentiation of mouse embryonic stem cells in vitro

Front Cell Neurosci

11

(33):

20170221

PMID:28270748

DOI: 10.3389/fncel.2017.00033

PMCID: 5318384

Long JD, | Langbehn DR, | Tabrizi SJ, | Landwehrmeyer BG, | Paulsen JS, | Warner J, | Sampaio C |

Validation of a prognostic index for Huntington's disease

Mov Disord

256-263

32

(2):

20170220

PMID:27892614

DOI: 10.1002/mds.26838

PMCID: 5318276

Skene DJ, | Middleton B, | Fraser CK, | Pennings JL, | Kuchel TR, | Rudiger SR, | Bawden CS, | Morton AJ |

Metabolic profiling of presymptomatic Huntington's disease sheep reveals novel biomarkers

Sci Reports

1-16

7

(43030):

20170217

PMID:28223686

DOI: 10.1038/srep43030

PMCID: 5320451

Faedo A, | Laporta A, | Segnali A, | Galimberti M, | Besusso D, | Cesana E, | Belloli S, | Moresco RM, | Tropiano M, | Fucà E, | Wild S, | Bosio A, | Vercelli AE, | Biella G, | Cattaneo E |

Differentiation of human telencephalic progenitor cells into MSNs by inducible expression of Gsx2 and Ebf1

Proc Natl Acad Sci USA

E1234-E1242

114

(7):

20170214

PMID:28137879

DOI: 10.1073/pnas.1611473114

PMCID: 5321017

Shin A, | Shin B, | Shin JW, | Kim KH, | Atwal RS, | Hope JM, | Gillis T, | Leszyk JD, | Shaffer SA, | Lee R, | Kwak S, | MacDonald ME, | Gusella JF, | Seong IS, | Lee JM |

Novel allele-specific quantification methods reveal no effects of adult onset CAG repeats on HTT mRNA and protein levels

Hum Mol Genet

1258-1267

26

(7):

20170206

PMID:28165127

DOI: 10.1093/hmg/ddx033

PMCID:

Shankaran M, | Di Paolo E, | Leoni V, | Caccia C, | Ferrari Bardile C, | Mohammed H, | Di Donato S, | Kwak S, | Marchionini D, | Turner S, | Cattaneo E, | Valenza M |

Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice

Neurobiol Dis

66-76

98

():

20170201

PMID:27913290

DOI: 10.1016/j.nbd.2016.11.013

PMCID:

Maiuri T, | Mocle AJ, | Hung CL, | Xia J, | van Roon-Mom WM, | Truant R |

Huntingtin is a scaffolding protein in the ATM oxidative DNA damage response complex

Hum Mol Genet

395-406

26

(2):

20170115

PMID:28017939

DOI: 10.1093/hmg/ddw395

PMCID:

Lee JK, | Ding Y, | Conrad AL, | Cattaneo E, | Epping E, | Mathews K, | Gonzalez-Alegre P, | Cahill L, | Magnotta V, | Schlaggar BL, | Perlmutter JS, | Kim RE, | Dawson JD, | Nopoulos P |

Sex-specific effects of the Huntington gene on normal neurodevelopment

J Neurosci Res

398-408

95

(1-2):

20170102

PMID:27870408

DOI: 10.1002/jnr.23980

PMCID:

2016

Orth M, | Gregory S, | Scahill RI, | Mayer IS, | Minkova L, | Klöppel S, | Seunarine KK, | Boyd L, | Borowsky B, | Reilmann R, | Landwehrmeyer BG, | Leavitt BR, | Roos RA, | Durr A, | Rees G, | Rothwell JC, | Langbehn D, | Tabrizi SJ, | TRACK-On Investigators |

Natural variation in sensory-motor white matter organization influences manifestations of Huntington's disease

Hum Brain Mapp

4615-4628

37

(12):

20161231

PMID:27477323

DOI: 10.1002/hbm.23332

PMCID:

Beaumont V, | Zhong S, | Lin H, | Xu W, | Bradaia A, | Steidl E, | Gleyzes M, | Wadel K, | Buisson B, | Padovan-Neto FE, | Chakroborty S, | Ward KM, | Harms JF, | Beltran J, | Kwan M, | Ghavami A, | Häggkvist J, | Tóth M, | Halldin C, | Varrone A, | Schaab C, | Dybowski JN, | Elschenbroich S, | Lehtimäki K, | Heikkinen T, | Park L, | Rosinski J, | Mrzljak L, | Lavery D, | West AR, | Schmidt CJ, | Zaleska MM, | Munoz-Sanjuan I |

Phosphodiesterase 10A inhibition improves cortico-basal ganglia function in Huntington's disease models

Neuron

1220-1237

92

(6):

20161221

PMID:27916455

DOI: 10.1016/j.neuron.2016.10.064

PMCID:

Atherton JF, | McIver EL, | Mullen MR, | Wokosin DL, | Surmeier DJ, | Bevan MD |

Early dysfunction and progressive degeneration of the subthalamic nucleus in mouse models of Huntington's disease

Elife

e21616

5

():

20161220

PMID:27995895

DOI: 10.7554/eLife.21616

PMCID: 5199195

Deng YP, | Reiner A |

Cholinergic interneurons in the Q140 knockin mouse model of Huntington's disease: Reductions in dendritic branching and thalamostriatal input

J Comp Neurol

3518-3529

524

(17):

20161201

PMID:27219491

DOI: 10.1002/cne.24013

PMCID: 5050058

Keeler AM, | Sapp E, | Chase K, | Sottosanti E, | Danielson E, | Pfister E, | Stoica L, | DiFiglia M, | Aronin N, | Sena-Esteves M |

Cellular analysis of silencing the Huntington's disease gene using AAV9 mediated delivery of artificial micro RNA into the Striatum of Q140/Q140 mice

J Huntingtons Dis

239-248

5

(3):

20161001

PMID:27689620

DOI: 10.3233/JHD-160215

PMCID:

Verani M, | Bustamante M, | Martufi P, | Daldin M, | Cariulo C, | Azzollini L, | Fodale V, | Puglisi F, | Weiss A, | Macdonald D, | Petricca L, | Caricasole A |

Conformational modulation mediated by polyglutamine expansion in CAG repeat expansion disease-associated proteins

Biochem Biophys Res Commun

949-55

478

(2):

20160916

PMID:27520369

DOI: 10.1016/j.bbrc.2016.08.057

PMCID:

Naseri NN, | Bonica J, | Xu H, | Park LC, | Arjomand J, | Chen Z, | Gibson GE |

Novel metabolic abnormalities in the tricarboxylic acid cycle in peripheral cells from Huntington's disease patients

PLoS One

e0160384

11

(9):

20160909

PMID:27611087

DOI: 10.1371/journal.pone.0160384

PMCID: 5017661

Ruggeri FS, | Vieweg S, | Cendrowska U, | Longo G, | Chiki A, | Lashuel HA, | Dietler G |

Nanoscale studies link amyloid maturity with polyglutamine diseases onset

Sci Rep

31155

6

():

20160808

PMID:27499269

DOI: 10.1038/srep31155

PMCID: 4976327

Warner JH, | Sampaio C |

Modeling variability in the progression of Huntington's disease a novel modeling approach applied to structural imaging markers from TRACK-HD

CPT Pharmacometrics Syst Pharmacol

437-45

5

(8):

20160802

PMID:27481337

DOI: 10.1002/psp4.12097

PMCID: 4999607

Beaumont V, | Mrzljak L, | Dijkman U, | Freije R, | Heins M, | Rassoulpour A, | Tombaugh G, | Gelman S, | Bradaia A, | Steidl E, | Gleyzes M, | Heikkinen T, | Lehtimaki K, | Puolivali J, | Kontkanen O, | Javier RM, | Neagoe I, | Deisemann H, | Winkler D, | Ebneth A, | Khetarpal V, | Toledo-Sherman L, | Dominguez C, | Park LC, | Munoz-Sanjuan I |

The novel KMO inhibitor CHDI-340246 leads to a restoration of electrophysiological alterations in mouse models of Huntington's disease

Exp Neurol

99-118

282

():

20160801

PMID:27163548

DOI: 10.1016/j.expneurol.2016.05.005

PMCID:

Cockerham R, | Liu S, | Cachope R, | Kiyokage E, | Cheer JF, | Shipley MT, | Puche AC |

Subsecond regulation of synaptically released dopamine by COMT in the olfactory bulb

J Neurosci

7779-85

36

(29):

20160720

PMID:27445153

DOI: 10.1523/JNEUROSCI.0658-16.2016

PMCID: 4951580

Alexandrov V, | Brunner D, | Menalled LB, | Kudwa A, | Watson-Johnson J, | Mazzella M, | Russell I, | Ruiz MC, | Torello J, | Sabath E, | Sanchez A, | Gomez M, | Filipov I, | Cox K, | Kwan M, | Ghavami A, | Ramboz S, | Lager B, | Wheeler VC, | Aaronson J, | Rosinski J, | Gusella JF, | MacDonald ME, | Howland D, | Kwak S |

Large-scale phenome analysis defines a behavioral signature for Huntington's disease genotype in mice

Nat Biotechnol

838-44

34

(8):

20160704

PMID:27376585

DOI: 10.1038/nbt.3587

PMCID:

Horvath S, | Langfelder P, | Kwak S, | Aaronson J, | Rosinski J, | Vogt TF, | Eszes M, | Faull RL, | Curtis MA, | Waldvogel HJ, | Choi OW, | Tung S, | Vinters HV, | Coppola G, | Yang XW |

Huntington's disease accelerates epigenetic aging of human brain and disrupts DNA methylation levels

Aging (Albany NY)

1485-512

8

(7):

20160701

PMID:27479945

DOI: 10.18632/aging.101005

PMCID:

Labuschagne I, | Cassidy AM, | Scahill RI, | Johnson EB, | Rees E, | O'Regan A, | Queller S, | Frost C, | Leavitt BR, | Dürr A, | Roos R, | Owen G, | Borowsky B, | Tabrizi SJ, | Stout JC, | TRACK-HD Investigators |

Visuospatial processing deficits linked to posterior brain regions in premanifest and early stage Huntington's disease

J Int Neuropsychol Soc

595-608

22

(6):

20160701

PMID:27211109

DOI: 10.1017/S1355617716000321

PMCID:

Frich JC, | Rae D, | Roxburgh R, | Miedzybrodzka ZH, | Edmondson M, | Pope EB, | Goodman L, | Haddad MS, | Giuliano J, | Nelson EC, | Guttman M, | Nance M |

Health care delivery practices in Huntington's disease specialty clinics: an international survey

J Huntingtons Dis

207-13

5

(2):

20160627

PMID:27372053

DOI: 10.3233/JHD-160192

PMCID:

Landwehrmeyer GB, | Fitzer-Attas CJ, | Giuliano JD, | Gonçalves N, | Anderson KE, | Cardoso F, | Ferreira JJ, | Mestre TA, | Stout JC, | Sampaio C |

Data analytics from Enroll-HD, a global clinical research platform for Huntington's disease

Mov Disord Clin Pract

212-224

4

(2):

20160622

PMID:30363395

DOI: 10.1002/mdc3.12388

PMCID: 6174428

Hintiryan H, | Foster NN, | Bowman I, | Bay M, | Song MY, | Gou L, | Yamashita S, | Bienkowski MS, | Zingg B, | Zhu M, | Yang XW, | Shih JC, | Toga AW, | Dong HW |

The mouse cortico-striatal projectome

Nat Neurosci

1100-14

19

(8):

20160620

PMID:27322419

DOI: 10.1038/nn.4332

PMCID:

Mestre TA, | van Duijn E, | Davis AM, | Bachoud-Levi AC, | Busse M, | Anderson KE, | Ferreira JJ, | Mahlknecht P, | Tumas V, | Sampaio C, | Goetz CG, | Cubo E, | Stebbins GT, | Martinez-Martin P, | Members of the MDS Committee on Rating Scales Development |

Rating scales for behavioral symptoms in Huntington's disease: Critique and recommendations

Mov Disord

1466-1478

31

(10):

20160614

PMID:27296904

DOI: 10.1002/mds.26675

PMCID:

Vieweg S, | Ansaloni A, | Wang ZM, | Warner JB, | Lashuel HA |

An intein-based strategy for the production of tag-free Huntingtin Exon 1 proteins enables new insights into the polyglutamine dependence of Httex1 aggregation and fibril formation

J Biol Chem

12074-86

291

(23):

20160603

PMID:27002149

DOI: 10.1074/jbc.M116.713982

PMCID: 4933259

Schramke S, | Schuldenzucker V, | Schubert R, | Frank F, | Wirsig M, | Ott S, | Motlik J, | Fels M, | Kemper N, | Holzner E, | Reilmann R |

Behavioral phenotyping of minipigs transgenic for the Huntington gene

J Neurosci Methods

34-45

265

():

20160530

PMID:26688470

DOI: 10.1016/j.jneumeth.2015.11.013

PMCID:

Schubert R, | Frank F, | Nagelmann N, | Liebsch L, | Schuldenzucker V, | Schramke S, | Wirsig M, | Johnson H, | Kim EY, | Ott S, | Holzner E, | Demokritov SO, | Motlik J, | Faber C, | Reilmann R |

Neuroimaging of a minipig model of Huntington's disease: Feasibility of volumetric, diffusion-weighted and spectroscopic assessments

J Neurosci Methods

46-55

265

():

20160530

PMID:26658298

DOI: 10.1016/j.jneumeth.2015.11.017

PMCID:

Baker CR, | Dominguez DJF, | Stout JC, | Gabery S, | Churchyard A, | Chua P, | Egan GF, | Petersen A, | Georgiou-Karistianis N, | Poudel GR |

Subjective sleep problems in Huntington's disease: A pilot investigation of the relationship to brain structure, neurocognitive, and neuropsychiatric function

J Neurol Sci

148-53

364

():

20160515

PMID:27084236

DOI: 10.1016/j.jns.2016.03.021

PMCID:

Ochaba J, | Monteys AM, | O`Rourke JG, | Reidling JC, | Steffan JS, | Davidson BL, | Thompson LM |

PIAS1 regulates mutant Huntingtin accumulation and Huntington's disease-associated phenotypes in vivo

Neuron

507-20

90

(3):

20160504

PMID:27146268

DOI: 10.1016/j.neuron.2016.03.016

PMCID: 4942306

Zhang N, | Bailus BJ, | Ring KL, | Ellerby LM |

iPSC-based drug screening for Huntington's disease

Brain Res

42-56

1638

():

20160501

PMID:26428226

DOI: 10.1016/j.brainres.2015.09.020

PMCID: 4814369

Choudhury SR, | Harris AF, | Cabral DJ, | Keeler AM, | Sapp E, | Ferreira JS, | Gray-Edwards HL, | Johnson JA, | Johnson AK, | Su Q, | Stoica L, | DiFiglia M, | Aronin N, | Martin DR, | Gao G, | Sena-Esteves M |

Widespread central nervous system gene transfer and silencing after systemic delivery of novel AAV-AS vector

Mol Ther

726-35

24

(4):

20160429

PMID:26708003

DOI: 10.1038/mt.2015.231

PMCID: 4886933

Kumar A, | Zhang J, | Tallaksen-Greene S, | Crowley MR, | Crossman DK, | Morton AJ, | Van Groen T, | Kadish I, | Albin RL, | Lesort M, | Detloff PJ |

Allelic series of Huntington's disease knock-in mice reveals expression discorrelates

Hum Mol Genet

1619-36

25

(8):

20160415

PMID:26908599

DOI: 10.1093/hmg/ddw040

PMCID: 4805312

Langfelder P, | Cantle JP, | Chatzopoulou D, | Wang N, | Gao F, | Al-Ramahi I, | Lu XH, | Ramos EM, | El-Zein K, | Zhao Y, | Deverasetty S, | Tebbe A, | Schaab C, | Lavery DJ, | Howland D, | Kwak S, | Botas J, | Aaronson JS, | Rosinski J, | Coppola G, | Horvath S, | Yang XW |

Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice

Nat Neurosci

623-33

19

(4):

20160401

PMID:26900923

DOI: 10.1038/nn.4256

PMCID:

Baake V, | Hart EP, | Bos R, | Roos RA |

Participants at the Leiden site of the REGISTRY Study: a demographic approach

J Huntingtons Dis

83-90

5

(1):

20160315

PMID:27003663

DOI: 10.3233/JHD-150157

PMCID:

Munoz-Sanjuan I |

Support communities involved in disease studies

Nature

141

531

(7593):

20160310

PMID:26961621

DOI: 10.1038/531141a

PMCID:

Fisher SP, | Schwartz MD, | Wurts-Black S, | Thomas AM, | Chen TM, | Miller MA, | Palmerston JB, | Kilduff TS, | Morairty SR |

Quantitative electroencephalographic analysis provides an early-stage indicator of disease onset and progression in the zQ175 knock-in mouse model of Huntington's disease

Sleep

379-91

39

(2):

20160216

PMID:26446107

DOI: 10.5665/sleep.5448

PMCID: 4712407

Wiecki TV, | Antoniades CA, | Stevenson A, | Kennard C, | Borowsky B, | Owen G, | Leavitt B, | Roos R, | Durr A, | Tabrizi SJ, | Frank MJ |

A computational cognitive biomarker for early-stage Huntington's disease

PLoS One

e0148409

11

(2):

20160212

PMID:26872129

DOI: 10.1371/journal.pone.0148409

PMCID: 4752511

Keum JW, | Shin A, | Gillis T, | Mysore JS, | Abu Elneel K, | Lucente D, | Hadzi T, | Holmans P, | Jones L, | Orth M, | Kwak S, | MacDonald ME, | Gusella JF, | Lee JM |

The Htt CAG-expansion mutation determines age at death but not disease duration in Huntington disease

Am J Hum Genet

287-98

98

(2):

20160204

PMID:26849111

DOI: 10.1016/j.ajhg.2015.12.018

PMCID: 4746370

Nambron R, | Silajdžić E, | Kalliolia E, | Ottolenghi C, | Hindmarsh P, | Hill NR, | Costelloe SJ, | Martin NG, | Positano V, | Watt HC, | Frost C, | Björkqvist M, | Warner TT |

A metabolic study of Huntington's disease

PLoS One

e0146480

11

(1):

20160108

PMID:26744893

DOI: 10.1371/journal.pone.0146480

PMCID: 4706313

Carvalho JO, | Long JD, | Westervelt HJ, | Smith MM, | Bruce JM, | Kim JI, | Mills JA, | Paulsen JS, | PREDICT-HD Investigators and Coordinators | of The Huntington Study Group |

The impact of oculomotor functioning on neuropsychological performance in Huntington disease

J Clin Exp Neuropsychol

217-26

38

(2):

20160101

PMID:26745770

DOI: 10.1080/13803395.2015.1101054

PMCID:

Landwehrmeyer GB, | Fitzer-Attas CJ, | Giuliano JD, | Gonsalves N, | Anderson KE, | Cardoso F, | Ferreira JJ, | Mestre TA, | Stout JC, | Sampaio C |

Data analytics from Enroll-HD, a global clinical research platform for Huntington's Disease

Movement Disorders Clinical Practice

():

20160101

PMID:

DOI: 10.1002/mdc3.12388

PMCID:

Keogh R, | Frost C, | Owen G, | Daniel RM, | Langbehn DR, | Leavitt B, | Durr A, | Roos RA, | Landwehrmeyer GB, | Reilmann R, | Borowsky B, | Stout J, | Craufurd D, | Tabrizi SJ |

Medication use in early-HD participants in Track-HD: an investigation of its effects on clinical performance

PLoS Curr

8

():

20160101

PMID:26819833

DOI: 10.1371/currents.hd.8060298fac1801b01ccea6acc00f97cb

PMCID: 4718719

Culver BP, | DeClercq J, | Dolgalev I, | Yu MS, | Ma B, | Heguy A, | Tanese N |

Huntington's Disease protein Huntingtin associates with its own mRNA

J Huntingtons Dis

39-51

5

(1):

20160101

PMID:26891106

DOI: 10.3233/JHD-150177

PMCID: 4927879

Benraiss A, | Wang S, | Herrlinger S, | Li X, | Chandler-Militello D, | Mauceri J, | Burm HB, | Toner M, | Osipovitch M, | Jim Xu Q, | Ding F, | Wang F, | Kang N, | Kang J, | Curtin PC, | Brunner D, | Windrem MS, | Munoz-Sanjuan I, | Nedergaard M, | Goldman SA |

Human glia can both induce and rescue aspects of disease phenotype in Huntington disease

Nat Commun

1-13

7

():

20160101

PMID:27273432

DOI: 10.1038/ncomms11758

PMCID: 4899632

2015

Puigdellivol M, | Cherubini M, | Brito V, | Giralt A, | Suelves N, | Ballesteros J, | Zamora-Moratalla A, | Martin ED, | Eipper BA, | Alberch J, | Gines S |

A role for Kalirin-7 in corticostriatal synaptic dysfunction in Huntington's disease

Hum Mol Genet

7265-85

24

(25):

20151220

PMID:26464483

DOI: 10.1093/hmg/ddv426

PMCID: 4664166

Dominguez DJF, | Stout JC, | Poudel G, | Churchyard A, | Chua P, | Egan GF, | Georgiou-Karistianis N |

Multimodal imaging biomarkers in premanifest and early Huntington's disease: 30-month IMAGE-HD data

Br J Psychiatry

571-8

208

(6):

20151217

PMID:26678864

DOI: 10.1192/bjp.bp.114.156588

PMCID:

Luckhurst CA, | Breccia P, | Stott AJ, | Aziz O, | Birch HL, | Bürli RW, | Hughes SJ, | Jarvis RE, | Lamers M, | Leonard PM, | Matthews KL, | McAllister G, | Pollack S, | Saville-Stones E, | Wishart G, | Yates D, | Dominguez C |

Potent, selective, and CNS-penetrant tetrasubstituted cyclopropane class IIa Histone Deacetylase (HDAC) Inhibitors

ACS Med Chem Lett

34-9

7

(1):

20151210

PMID:26819662

DOI: 10.1021/acsmedchemlett.5b00302

PMCID: 4716601

Ring KL, | An MC, | Zhang N, | O'Brien RN, | Ramos EM, | Gao F, | Atwood R, | Bailus BJ, | Melov S, | Mooney SD, | Coppola G, | Ellerby LM |

Genomic analysis reveals disruption of striatal neuronal development and therapeutic targets in human Huntington's disease neural stem cells

Stem Cell Reports

1023-1038

5

(6):

20151208

PMID:26651603

DOI: 10.1016/j.stemcr.2015.11.005

PMCID: 4682390

Dickey AS, | Pineda VV, | Tsunemi T, | Liu PP, | Miranda HC, | Gilmore-Hall SK, | Lomas N, | Sampat KR, | Buttgereit A, | Torres MM, | Flores AL, | Arreola M, | Arbez N, | Akimov SS, | Gaasterland T, | Lazarowski ER, | Ross CA, | Yeo GW, | Sopher BL, | Magnuson GK, | Pinkerton AB, | Masliah E, | La Spada AR |

PPAR-delta is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically

Nat Med

37-45

22

(1):

20151207

PMID:26642438

DOI: 10.1038/nm.4003

PMCID:

Banez-Coronel M, | Ayhan F, | Tarabochia AD, | Zu T, | Perez BA, | Tusi SK, | Pletnikova O, | Borchelt DR, | Ross CA, | Margolis RL, | Yachnis AT, | Troncoso JC, | Ranum LP |

RAN translation in Huntington disease

Neuron

667-77

88

(4):

20151118

PMID:26590344

DOI: 10.1016/j.neuron.2015.10.038

PMCID: 4684947

Liu WC, | Kohn J, | Szwed SK, | Pariser E, | Sepe S, | Haripal B, | Oshimori N, | Marsala M, | Miyanohara A, | Lee R |

Human mutant huntingtin disrupts vocal learning in transgenic songbirds

Nat Neurosci

1617-22

18

(11):

20151101

PMID:26436900

DOI: 10.1038/nn.4133

PMCID:

McColgan P, | Seunarine KK, | Razi A, | Cole JH, | Gregory S, | Durr A, | Roos RA, | Stout JC, | Landwehrmeyer B, | Scahill RI, | Clark CA, | Rees G, | Tabrizi SJ, | Track-HD Investigators |

Selective vulnerability of Rich Club brain regions is an organizational principle of structural connectivity loss in Huntington's disease

Brain

3327-44

138

(Pt 11):

20151101

PMID:26384928

DOI: 10.1093/brain/awv259

PMCID:

Politis M, | Lahiri N, | Niccolini F, | Su P, | Wu K, | Giannetti P, | Scahill RI, | Turkheimer FE, | Tabrizi SJ, | Piccini P |

Increased central microglial activation associated with peripheral cytokine levels in premanifest Huntington's disease gene carriers

Neurobiol Dis

115-21

83

():

20151101

PMID:26297319

DOI: 10.1016/j.nbd.2015.08.011

PMCID:

Liu WC, | Kohn J, | Szwed SK, | Pariser E, | Sepe S, | Haripal B, | Oshimori N, | Marsala M, | Miyanohara A, | Lee R |

Human mutant huntingtin disrupts vocal learning in transgenic songbirds

Nat Neurosci

1617-22

18

(11):

20151101

PMID:26436900

DOI: 10.1038/nn.4133

PMCID:

Epping EA, | Kim JI, | Craufurd D, | Brashers-Krug TM, | Anderson KE, | McCusker E, | Luther J, | Long JD, | Paulsen JS, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

Longitudinal psychiatric symptoms in prodromal Huntington's disease: a decade of data

Am J Psychiatry

184-92

173

(2):

20151016

PMID:26472629

DOI: 10.1176/appi.ajp.2015.14121551

PMCID:

Minkova L, | Eickhoff SB, | Abdulkadir A, | Kaller CP, | Peter J, | Scheller E, | Lahr J, | Roos RA, | Durr A, | Leavitt BR, | Tabrizi SJ, | Kloppel S, | Track-HD Investigators |

Large-scale brain network abnormalities in Huntington's disease revealed by structural covariance

Hum Brain Mapp

67-80

37

(1):

20151010

PMID:26453902

DOI: 10.1002/hbm.23014

PMCID:

Kim JI, | Long JD, | Mills JA, | Downing N, | Williams JK, | Paulsen JS, | Predict-HD Investigators, | Coordinators of the Huntington Study Group |

Performance of the 12-item WHODAS 2.0 in prodromal Huntington disease

Eur J Hum Genet

1584-7

23

(11):

20151001

PMID:25735480

DOI: 10.1038/ejhg.2015.11

PMCID:

Lazar AS, | Panin F, | Goodman AO, | Lazic SE, | Lazar ZI, | Mason SL, | Rogers L, | Murgatroyd PR, | Watson LP, | Singh P, | Borowsky B, | Shneerson JM, | Barker RA |

Sleep deficits but no metabolic deficits in premanifest Huntington's disease

Ann Neurol

630-48

78

(4):

20151001

PMID:26224419

DOI: 10.1002/ana.24495

PMCID:

Matsui JT, | Vaidya JG, | Wassermann D, | Kim RE, | Magnotta VA, | Johnson HJ, | Paulsen JS, | Predict-HD Investigators, | Coordinators of the Huntington Study Group |

Prefrontal cortex white matter tracts in prodromal Huntington disease

Hum Brain Mapp

3717-32

36

(10):

20151001

PMID:26179962

DOI: 10.1002/hbm.22835

PMCID: 4583330

Long JD, | Paulsen JS, | Predict-HD Investigators, | Coordinators of the Huntington Study Group |

Multivariate prediction of motor diagnosis in Huntington's disease: 12 years of PREDICT-HD

Mov Disord

1664-72

30

(12):

20151001

PMID:26340420

DOI: 10.1002/mds.26364

PMCID:

Beglinger LJ, | Adams WH, | Fiedorowicz JG, | Duff K, | Langbehn D, | Biglan K, | Caviness J, | Olson B, | Paulsen JS |

Practice effects and stability of neuropsychological and UHDRS tests over short retest intervals in Huntington disease

J Huntingtons Dis

251-60

4

(3):

20150929

PMID:26444022

DOI: 10.3233/JHD-150159

PMCID:

Johnson E, | Chase K, | McGowan S, | Mondo E, | Pfister E, | Mick E, | Friedline RH, | Kim JK, | Sapp E, | DiFiglia M, | Aronin N |

Safety of striatal infusion of siRNA in a transgenic Huntington's disease mouse model

J Huntingtons Dis

219-29

4

(3):

20150929

PMID:26444021

DOI: 10.3233/JHD-150163

PMCID:

Correia K, | Harold D, | Kim KH, | Holmans P, | Jones L, | Orth M, | Myers RH, | Kwak S, | Wheeler VC, | MacDonald ME, | Gusella JF, | Lee JM |

The Genetic Modifiers of Motor OnsetAge (GeM MOA) website: Genome-wide association analysis for genetic modifiers of Huntington's disease

J Huntingtons Dis

279-84

4

(3):

20150929

PMID:26444025

DOI: 10.3233/JHD-150169

PMCID:

Gregory S, | Scahill RI, | Seunarine KK, | Stopford C, | Zhang H, | Zhang J, | Orth M, | Durr A, | Roos RA, | Langbehn DR, | Long JD, | Johnson H, | Rees G, | Tabrizi SJ, | Craufurd D |

Neuropsychiatry and white matter microstructure in Huntington's disease

J Huntingtons Dis

239-49

4

(3):

20150929

PMID:26443926

DOI: 10.3233/JHD-150160

PMCID: 4684097

Liu D, | Long JD, | Zhang Y, | Raymond LA, | Marder K, | Rosser A, | McCusker EA, | Mills JA, | Paulsen JS, | Predict-HD Investigators, | Coordinators of the Huntington Study Group |

Motor onset and diagnosis in Huntington disease using the diagnostic confidence level

J Neurol

2691-8

262

(12):

20150926

PMID:26410751

DOI: 10.1007/s00415-015-7900-7

PMCID:

Straccia M, | Garcia-Diaz Barriga G, | Sanders P, | Bombau G, | Carrere J, | Mairal PB, | Vinh NN, | Yung S, | Kelly CM, | Svendsen CN, | Kemp PJ, | Arjomand J, | Schoenfeld RC, | Alberch J, | Allen ND, | Rosser AE, | Canals JM |

Quantitative high-throughput gene expression profiling of human striatal development to screen stem cell-derived medium spiny neurons

Mol Ther Methods Clin Dev

15030

2

():

20150916

PMID:26417608

DOI: 10.1038/mtm.2015.30

PMCID: 4571731

Gatto RG, | Chu Y, | Ye AQ, | Price SD, | Tavassoli E, | Buenaventura A, | Brady ST, | Magin RL, | Kordower JH, | Morfini GA |

Analysis of YFP(J16)-R6/2 reporter mice and postmortem brains reveals early pathology and increased vulnerability of callosal axons in Huntington's disease

Hum Mol Genet

5285-98

24

(18):

20150915

PMID:26123489

DOI: 10.1093/hmg/ddv248

PMCID: 4550824

Ciamei A, | Detloff PJ, | Morton AJ |

Progression of behavioural despair in R6/2 and Hdh knock-in mouse models recapitulates depression in Huntington's disease

Behav Brain Res

140-6

291

():

20150915

PMID:25986402

DOI: 10.1016/j.bbr.2015.05.010

PMCID:

Bustamante MB, | Ansaloni A, | Pedersen JF, | Azzollini L, | Cariulo C, | Wang ZM, | Petricca L, | Verani M, | Puglisi F, | Park H, | Lashuel H, | Caricasole A |

Detection of huntingtin exon 1 phosphorylation by Phos-Tag SDS-PAGE: Predominant phosphorylation on threonine 3 and regulation by IKKbeta

Biochem Biophys Res Commun

1317-22

463

(4):

20150807

PMID:26106822

DOI: 10.1016/j.bbrc.2015.06.116

PMCID:

Harrington DL, | Rubinov M, | Durgerian S, | Mourany L, | Reece C, | Koenig K, | Bullmore E, | Long JD, | Paulsen JS, | Predict-Hd investigators of the Huntington Study Group, | Rao SM |

Network topology and functional connectivity disturbances precede the onset of Huntington's disease

Brain

2332-46

138

(Pt 8):

20150801

PMID:26059655

DOI: 10.1093/brain/awv145

PMCID:

Plotkin JL, | Surmeier DJ |

Corticostriatal synaptic adaptations in Huntington's disease

Curr Opin Neurobiol

53-62

33

():

20150801

PMID:25700146

DOI: 10.1016/j.conb.2015.01.020

PMCID:

Arrowsmith CH, | Audia JE, | Austin C, | Baell J, | Bennett J, | Blagg J, | Bountra C, | Brennan PE, | Brown PJ, | Bunnage ME, | Buser-Doepner C, | Campbell RM, | Carter AJ, | Cohen P, | Copeland RA, | Cravatt B, | Dahlin JL, | Dhanak D, | Edwards AM, | Frye SV, | Gray N, | Grimshaw CE, | Hepworth D, | Howe T, | Huber KV, | Jin J, | Knapp S, | Kotz JD, | Kruger RG, | Lowe D, | Mader MM, | Marsden B, | Mueller-Fahrnow A, | Muller S, | O`Hagan RC, | Overington JP, | Owen DR, | Rosenberg SH, | Roth B, | Ross R, | Schapira M, | Schreiber SL, | Shoichet B, | Sundstrom M, | Superti-Furga G, | Taunton J, | Toledo-Sherman L, | Walpole C, | Walters MA, | Willson TM, | Workman P, | Young RN, | Zuercher WJ |

The promise and peril of chemical probes

Nat Chem Biol

536-41

11

(8):

20150801

PMID:26196764

DOI: 10.1038/nchembio.1867

PMCID:

O`Brien R, | DeGiacomo F, | Holcomb J, | Bonner A, | Ring KL, | Zhang N, | Zafar K, | Weiss A, | Lager B, | Schilling B, | Gibson BW, | Chen S, | Kwak S, | Ellerby LM |

Integration-independent transgenic Huntington disease fragment mouse models reveal distinct phenotypes and life span in vivo

J Biol Chem

19287-306

290

(31):

20150731

PMID:26025364

DOI: 10.1074/jbc.M114.623561

PMCID: 4521048

GeM-HD Consortium |

Identification of genetic factors that modify clinical onset of Huntington's disease

Cell

516-26

162

(3):

20150730

PMID:26232222

DOI: 10.1016/j.cell.2015.07.003

PMCID: 4524551

Khakh BS, | Sofroniew MV |

Diversity of astrocyte functions and phenotypes in neural circuits

Nat Neurosci

942-52

18

(7):

20150701

PMID:26108722

DOI: 10.1038/nn.4043

PMCID:

Achour M, | Le Gras S, | Keime C, | Parmentier F, | Lejeune FX, | Boutillier AL, | Néri C, | Davidson I, | Merienne K |

Neuronal identity genes regulated by super-enhancers are preferentially down-regulated in the striatum of Huntington's disease mice

Hum Mol Genet

3481-96

24

(12):

20150615

PMID:25784504

DOI: 10.1093/hmg/ddv099

PMCID:

Friedman A, | Homma D, | Gibb LG, | Amemori K, | Rubin SJ, | Hood AS, | Riad MH, | Graybiel AM |

A corticostriatal path targeting striosomes controls decision-making under conflict

Cell

1320-33

161

(6):

20150604

PMID:26027737

DOI: 10.1016/j.cell.2015.04.049

PMCID: 4477966

Odish OF, | Leemans A, | Reijntjes RH, | van den Bogaard SJ, | Dumas EM, | Wolterbeek R, | Tax CM, | Kuijf HJ, | Vincken KL, | van der Grond J, | Roos RA |

Microstructural brain abnormalities in Huntington's disease: A two-year follow-up

Hum Brain Mapp

2061-74

36

(6):

20150601

PMID:25644819

DOI: 10.1002/hbm.22756

PMCID:

Becanovic K, | Norremolle A, | Neal SJ, | Kay C, | Collins JA, | Arenillas D, | Lilja T, | Gaudenzi G, | Manoharan S, | Doty CN, | Beck J, | Lahiri N, | Portales-Casamar E, | Warby SC, | Connolly C, | De Souza RA, | Registry Investigators of the European Huntington`s Disease Network, | Tabrizi SJ, | Hermanson O, | Langbehn DR, | Hayden MR, | Wasserman WW, | Leavitt BR |

A SNP in the HTT promoter alters NF-kappaB binding and is a bidirectional genetic modifier of Huntington disease

Nat Neurosci

807-16

18

(6):

20150601

PMID:25938884

DOI: 10.1038/nn.4014

PMCID:

Dominguez JF, | Ng AC, | Poudel G, | Stout JC, | Churchyard A, | Chua P, | Egan GF, | Georgiou-Karistianis N |

Iron accumulation in the basal ganglia in Huntington's disease: cross-sectional data from the IMAGE-HD study

J Neurol Neurosurg Psychiatry

545-9

87

(5):

20150507

PMID:25952334

DOI: 10.1136/jnnp-2014-310183

PMCID:

Biagioli M, | Ferrari F, | Mendenhall EM, | Zhang Y, | Erdin S, | Vijayvargia R, | Vallabh SM, | Solomos N, | Manavalan P, | Ragavendran A, | Ozsolak F, | Lee JM, | Talkowski ME, | Gusella JF, | Macdonald ME, | Park PJ, | Seong IS |

Htt CAG repeat expansion confers pleiotropic gains of mutant huntingtin function in chromatin regulation

Hum Mol Genet

2442-57

24

(9):

20150501

PMID:25574027

DOI: 10.1093/hmg/ddv006

PMCID: 4383859

Novak MJ, | Seunarine KK, | Gibbard CR, | McColgan P, | Draganski B, | Friston K, | Clark CA, | Tabrizi SJ |

Basal ganglia-cortical structural connectivity in Huntington's disease

Hum Brain Mapp

1728-40

36

(5):

20150501

PMID:25640796

DOI: 10.1002/hbm.22733

PMCID:

Jin J, | Peng Q, | Hou Z, | Jiang M, | Wang X, | Langseth AJ, | Tao M, | Barker PB, | Mori S, | Bergles DE, | Ross CA, | Detloff PJ, | Zhang J, | Duan W |

Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease

Hum Mol Genet

2508-27

24

(9):

20150501

PMID:25609071

DOI: 10.1093/hmg/ddv016

PMCID: 4383863

Wild EJ, | Boggio R, | Langbehn D, | Robertson N, | Haider S, | Miller JR, | Zetterberg H, | Leavitt BR, | Kuhn R, | Tabrizi SJ, | Macdonald D, | Weiss A |

Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients

J Clin Invest

1979-86

125

(5):

20150501

PMID:25844897

DOI: 10.1172/JCI80743

PMCID: 4463213

Wityak J, | McGee KF, | Conlon MP, | Song RH, | Duffy BC, | Clayton B, | Lynch M, | Wang G, | Freeman E, | Haber J, | Kitchen DB, | Manning DD, | Ismail J, | Khmelnitsky Y, | Michels P, | Webster J, | Irigoyen M, | Luche M, | Hultman M, | Bai M, | Kuok ID, | Newell R, | Lamers M, | Leonard P, | Yates D, | Matthews K, | Ongeri L, | Clifton S, | Mead T, | Deupree S, | Wheelan P, | Lyons K, | Wilson C, | Kiselyov A, | Toledo-Sherman L, | Beconi M, | Muñoz-Sanjuan I, | Bard J, | Dominguez C |

Lead optimization toward proof-of-concept tools for Huntington's disease within a 4-(1H-pyrazol-4-yl) pyrimidine class of pan-JNK inhibitors

J Med Chem

2967-87

58

(7):

20150409

PMID:25760409

DOI: 10.1021/jm5013598

PMCID:

Valenza M, | Marullo M, | Di Paolo E, | Cesana E, | Zuccato C, | Biella G, | Cattaneo E |

Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington's disease

Cell Death Differ

690-702

22

(4):

20150401

PMID:25301063

DOI: 10.1038/cdd.2014.162

PMCID: 4356339

Sanders SS, | Hayden MR |

Aberrant palmitoylation in Huntington disease

Biochem Soc Trans

205-10

43

(2):

20150401

PMID:25849918

DOI: 10.1042/BST20140242

PMCID:

Khakh BS, | McCarthy KD |

Astrocyte calcium signaling: from observations to functions and the challenges therein

Cold Spring Harb Perspect Biol

a020404

7

(4):

20150401

PMID:25605709

DOI: 10.1101/cshperspect.a020404

PMCID:

Hering T, | Birth N, | Taanman JW, | Orth M |

Selective striatal mtDNA depletion in end-stage Huntington's disease R6/2 mice

Exp Neurol

22-9

266

():

20150401

PMID:25682918

DOI: 10.1016/j.expneurol.2015.02.004

PMCID:

Wassef SN, | Wemmie J, | Johnson CP, | Johnson H, | Paulsen JS, | Long JD, | Magnotta VA |

T1rho imaging in premanifest Huntington disease reveals changes associated with disease progression

Mov Disord

1107-14

30

(8):

20150329

PMID:25820773

DOI: 10.1002/mds.26203

PMCID:

Orsatti L, | Speziale R, | Orsale MV, | Caretti F, | Veneziano M, | Zini M, | Monteagudo E, | Lyons K, | Beconi M, | Chan K, | Herbst T, | Toledo-Sherman L, | Munoz-Sanjuan I, | Bonelli F, | Dominguez C |

A single-run liquid chromatography mass spectrometry method to quantify neuroactive kynurenine pathway metabolites in rat plasma

J Pharm Biomed Anal

426-31

107

():

20150325

PMID:25668794

DOI: 10.1016/j.jpba.2015.01.030

PMCID:

Penrose SD, | Stott AJ, | Breccia P, | Haughan AF, | Burli RW, | Jarvis RE, | Dominguez C |

Inter- and intramolecular annulation strategies to a cyclopentanone building block containing an all-carbon quaternary stereogenic center

Org Lett

1401-4

17

(6):

20150320

PMID:25720587

DOI: 10.1021/acs.orglett.5b00207

PMCID:

Estrada-Sanchez AM, | Burroughs CL, | Cavaliere S, | Barton SJ, | Chen S, | Yang XW, | Rebec GV |

Cortical efferents lacking mutant huntingtin improve striatal neuronal activity and behavior in a conditional mouse model of Huntington's disease

J Neurosci

4440-51

35

(10):

20150311

PMID:25762686

DOI: 10.1523/JNEUROSCI.2812-14.2015

PMCID: 4355206

Sturrock A, | Laule C, | Wyper K, | Milner RA, | Decolongon J, | Dar Santos R, | Coleman AJ, | Carter K, | Creighton S, | Bechtel N, | Bohlen S, | Reilmann R, | Johnson HJ, | Hayden MR, | Tabrizi SJ, | Mackay AL, | Leavitt BR |

A longitudinal study of magnetic resonance spectroscopy Huntington's disease biomarkers

Mov Disord

393-401

30

(3):

20150301

PMID:25690257

DOI: 10.1002/mds.26118

PMCID:

Cao Y, | Bartolomé-Martín D, | Rotem N, | Rozas C, | Dellal SS, | Chacon MA, | Kadriu B, | Gulinello M, | Khodakhah K, | Faber DS |

Rescue of homeostatic regulation of striatal excitability and locomotor activity in a mouse model of Huntington's disease

Proc Natl Acad Sci USA

2239-44

112

(7):

20150217

PMID:25646456

DOI: 10.1073/pnas.1405748112

PMCID: 4343133

Toledo-Sherman LM, | Prime ME, | Mrzljak L, | Beconi MG, | Beresford A, | Brookfield FA, | Brown CJ, | Cardaun I, | Courtney SM, | Dijkman U, | Hamelin-Flegg E, | Johnson PD, | Kempf V, | Lyons K, | Matthews K, | Mitchell WL, | O`Connell C, | Pena P, | Powell K, | Rassoulpour A, | Reed L, | Reindl W, | Selvaratnam S, | Friley WW, | Weddell DA, | Went NE, | Wheelan P, | Winkler C, | Winkler D, | Wityak J, | Yarnold CJ, | Yates D, | Munoz-Sanjuan I, | Dominguez C |

Development of a series of aryl pyrimidine kynurenine monooxygenase inhibitors as potential therapeutic agents for the treatment of Huntington's disease

J Med Chem

1159-83

58

(3):

20150212

PMID:25590515

DOI: 10.1021/jm501350y

PMCID:

Poudel GR, | Stout JC, | Dominguez DJ F, | Churchyard A, | Chua P, | Egan GF, | Georgiou-Karistianis N |

Longitudinal change in white matter microstructure in Huntington's disease: The IMAGE-HD study

Neurobiol Dis

406-12

74

():

20150201

PMID:25497085

DOI: 10.1016/j.nbd.2014.12.009

PMCID:

Dietz KN, | Di Stefano L, | Maher RC, | Zhu H, | Macdonald ME, | Gusella JF, | Walker JA |

The Drosophila Huntington's disease gene ortholog dhtt influences chromatin regulation during development

Hum Mol Genet

330-45

24

(2):

20150115

PMID:25168387

DOI: 10.1093/hmg/ddu446

PMCID:

Wood NI, | Morton AJ |

Social behaviour is impaired in the R6/2 mouse model of Huntington's disease

J Huntingtons Dis

61-73

4

(1):

20150101

PMID:26333258

DOI: 10.3233/JHD-140132

PMCID:

Oosterloo M, | Van Belzen MJ, | Bijlsma EK, | Roos RA |

Is there convincing evidence that intermediate repeats in the HTT gene cause Huntington's disease?

J Huntingtons Dis

141-8

4

(2):

20150101

PMID:26397895

DOI: 10.3233/JHD-140120

PMCID:

Poudel GR, | Stout JC, | Dominguez DJ, | Gray MA, | Salmon L, | Churchyard A, | Chua P, | Borowsky B, | Egan GF, | Georgiou-Karistianis N |

Functional changes during working memory in Huntington's disease: 30-month longitudinal data from the IMAGE-HD study

Brain Struct Funct

501-12

220

(1):

20150101

PMID:24240602

DOI: 10.1007/s00429-013-0670-z

PMCID:

Carroll JB, | Bates GP, | Steffan J, | Saft C, | Tabrizi SJ |

Treating the whole body in Huntington's disease

The Lancet Neurology

1135-1142

14

(11):

20150101

PMID:26466780

DOI: 10.1016/s1474-4422(15)00177-5

PMCID:

Odish OF, | van den Berg-Huysmans AA, | van den Bogaard SJ, | Dumas EM, | Hart EP, | Rombouts SA, | van der Grond J, | Roos RA, | Track-Hd Investigator Group |

Longitudinal resting state fMRI analysis in healthy controls and premanifest Huntington's disease gene carriers: a three-year follow-up study

Hum Brain Mapp

110-9

36

(1):

20150101

PMID:25139578

DOI: 10.1002/hbm.22616

PMCID:

Gabery S, | Georgiou-Karistianis N, | Lundh SH, | Cheong RY, | Churchyard A, | Chua P, | Stout JC, | Egan GF, | Kirik D, | Petersen A |

Volumetric analysis of the hypothalamus in Huntington Disease using 3T MRI: the IMAGE-HD Study

PLoS One

e0117593

10

(2):

20150101

PMID:25659157

DOI: 10.1371/journal.pone.0117593

PMCID: 4319930

Callaghan J, | Stopford C, | Arran N, | Boisse MF, | Coleman A, | Santos RD, | Dumas EM, | Hart EP, | Justo D, | Owen G, | Read J, | Say MJ, | Durr A, | Leavitt BR, | Roos RA, | Tabrizi SJ, | Bachoud-Levi AC, | Bourdet C, | van Duijn E, | Craufurd D |

Reliability and factor structure of the Short Problem Behaviors Assessment for Huntington's disease (PBA-s) in the TRACK-HD and REGISTRY studies

J Neuropsychiatry Clin Neurosci

59-64

27

(1):

20150101

PMID:25716488

DOI: 10.1176/appi.neuropsych.13070169

PMCID:

Kloppel S, | Gregory S, | Scheller E, | Minkova L, | Razi A, | Durr A, | Roos RAC, | Leavitt BR, | Papoutsi M, | Landwehrmeyer GB, | Reilmann R, | Borowsky B, | Johnson H, | Mills JA, | Owen G, | Stout J, | Scahill RI, | Long JD, | Rees G, | Tabrizi SJ |

Compensation in preclinical Huntington's disease: evidence from the Track-On HD study

E Bio Medicine

1420-9

2

(10):

20150101

PMID:26629536

DOI: 10.1016/j.ebiom.2015.08.002

PMCID:

Odish OF, | Caeyenberghs K, | Hosseini H, | van den Bogaard SJ, | Roos RA, | Leemans A |

Dynamics of the connectome in Huntington's disease: A longitudinal diffusion MRI study

Neuroimage Clin

32-43

9

():

20150101

PMID:26288754

DOI: 10.1016/j.nicl.2015.07.003

PMCID: 4536305

Mrzljak L, | Munoz-Sanjuan I |

Therapeutic strategies for Huntington's Disease

Curr Top Behav Neurosci

161-201

22

():

20150101

PMID:24277342

DOI: 10.1007/7854_2013_250

PMCID:

Reilmann R, | Lippross V, | Holzner E, | Gigengack F, | Bohlen S, | Kugel H, | Deppe M, | Osada N, | Lucke M, | Riess O, | Nguyen HP, | Von Horsten S, | Schafers K, | Schafers M, | Jacobs AH, | Hermann S |

FDG uPET fails to detect a disease-specific phenotype in rats transgenic for Huntington's disease

J Huntingtons Dis

37-47

4

(1):

20150101

PMID:26333256

DOI: 10.3233/JHD-130084

PMCID:

Naseri NN, | Xu H, | Bonica J, | Vonsattel JP, | Cortes EP, | Park LC, | Arjomand J, | Gibson GE |

Abnormalities in the tricarboxylic Acid cycle in Huntington disease and in a Huntington disease mouse model

J Neuropathol Exp Neurol

527-37

74

(6):

20150101

PMID:25978848

DOI: 10.1097/NEN.0000000000000197

PMCID: 4435838

Musso M, | Westervelt HJ, | Long JD, | Morgan E, | Woods SP, | Smith MM, | Lu W, | Paulsen JS, | Predict-HD Investigators of the Huntington Study Group |

Intra-individual variability in prodromal Huntington disease and its relationship to genetic burden

J Int Neuropsychol Soc

8-21

21

(1):

20150101

PMID:26304055

DOI: 10.1017/S1355617714001076

PMCID: 4549971

Curtin PC, | Farrar AM, | Oakeshott S, | Sutphen J, | Berger J, | Mazzella M, | Cox K, | He D, | Alosio W, | Park LC, | Howland D, | Brunner D |

Cognitive training at a young age attenuates deficits in the zQ175 mouse model of HD

Front Behav Neurosci

361

9

():

20150101

PMID:26793080

DOI: 10.3389/fnbeh.2015.00361

PMCID: 4707270

Alterman JF, | Hall LM, | Coles AH, | Hassler MR, | Didiot MC, | Chase K, | Abraham J, | Sottosanti E, | Johnson E, | Sapp E, | Osborn MF, | Difiglia M, | Aronin N, | Khvorova A |

Hydrophobically modified siRNAs silence huntingtin mRNA in primary neurons and mouse brain

Mol Ther Nucleic Acids

e266

4

():

20150101

PMID:26623938

DOI: 10.1038/mtna.2015.38

PMCID:

Cummings J, | Mintzer J, | Brodaty H, | Sano M, | Banerjee S, | Devanand DP, | Gauthier S, | Howard R, | Lanctot K, | Lyketsos CG, | Peskind E, | Porsteinsson AP, | Reich E, | Sampaio C, | Steffens D, | Wortmann M, | Zhong K, | International Psychogeriatric Association |

Agitation in cognitive disorders: International Psychogeriatric Association provisional consensus clinical and research definition

Int Psychogeriatr

7-17

27

(1):

20150101

PMID:25311499

DOI: 10.1017/S1041610214001963

PMCID: 4301197

Carty N, | Berson N, | Tillack K, | Thiede C, | Scholz D, | Kottig K, | Sedaghat Y, | Gabrysiak C, | Yohrling G, | von der Kammer H, | Ebneth A, | Mack V, | Munoz-Sanjuan I, | Kwak S |

Characterization of HTT inclusion size, location, and timing in the zQ175 mouse model of Huntingtons disease: an in vivo high-content imaging study

PLoS One

e0123527

10

(4):

20150101

PMID:25859666

DOI: 10.1371/journal.pone.0123527

PMCID: 4393127

Grondin R, | Ge P, | Chen Q, | Sutherland JE, | Zhang Z, | Gash DM, | Stiles DK, | Stewart GR, | Sah DW, | Kaemmerer WF |

Onset time and durability of Huntingtin suppression in Rhesus putamen after direct infusion of Antihuntingtin siRNA

Mol Ther Nucleic Acids

e245

4

():

20150101

PMID:26125484

DOI: 10.1038/mtna.2015.20

PMCID: 4561652

Carroll JB, | Deik A, | Fossale E, | Weston RM, | Guide JR, | Arjomand J, | Kwak S, | Clish CB, | MacDonald ME |

HdhQ111 mice exhibit tissue specific metabolite profiles that include striatal lipid accumulation

PLoS One

e0134465

10

(8):

20150101

PMID:26295712

DOI: 10.1371/journal.pone.0134465

PMCID: 4546654

2014

Paulsen JS, | Long JD, | Ross CA, | Harrington DL, | Erwin CJ, | Williams JK, | Westervelt HJ, | Johnson HJ, | Aylward EH, | Zhang Y, | Bockholt HJ, | Barker RA, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study

Lancet Neurol

1193-201

13

(12):

20141201

PMID:25453459

DOI: 10.1016/S1474-4422(14)70238-8

PMCID: 4373455

Yao J, | Ong SE, | Bajjalieh S |

Huntingtin is associated with cytomatrix proteins at the presynaptic terminal

Mol Cell Neurosci

96-100

63

():

20141101

PMID:25461618

DOI: 10.1016/j.mcn.2014.10.003

PMCID:

Deng YP, | Wong T, | Wan JY, | Reiner A |

Differential loss of thalamostriatal and corticostriatal input to striatal projection neuron types prior to overt motor symptoms in the Q140 knock-in mouse model of Huntington's disease

Front Syst Neurosci

198

8

():

20141015

PMID:25360089

DOI: 10.3389/fnsys.2014.00198

PMCID: 4197654

Sampaio C, | Borowsky B, | Reilmann R |

Clinical trials in Huntington's disease: Interventions in early clinical development and newer methodological approaches

Mov Disord

1419-28

29

(11):

20140915

PMID:25216371

DOI: 10.1002/mds.26021

PMCID:

Howland DS, | Munoz-Sanjuan I |

Mind the gap: models in multiple species needed for therapeutic development in Huntington's disease

Mov Disord

1397-403

29

(11):

20140915

PMID:25155258

DOI: 10.1002/mds.26008

PMCID:

Stout JC, | Queller S, | Baker KN, | Cowlishaw S, | Sampaio C, | Fitzer-Attas C, | Borowsky B, | HD-Cab Investigators |

HD-CAB: a cognitive assessment battery for clinical trials in Huntington's disease 1,2,3

Mov Disord

1281-8

29

(10):

20140901

PMID:25209258

DOI: 10.1002/mds.25964

PMCID:

Munafo M, | Noble S, | Browne WJ, | Brunner D, | Button K, | Ferreira J, | Holmans P, | Langbehn D, | Lewis G, | Lindquist M, | Tilling K, | Wagenmakers EJ, | Blumenstein R |

Scientific rigor and the art of motorcycle maintenance

Nat Biotechnol

871-3

32

(9):

20140901

PMID:25203032

DOI: 10.1038/nbt.3004

PMCID:

Smith GA, | Rocha EM, | McLean JR, | Hayes MA, | Izen SC, | Isacson O, | Hallett PJ |

Progressive axonal transport and synaptic protein changes correlate with behavioral and neuropathological abnormalities in the heterozygous Q175 KI mouse model of Huntington's disease

Hum Mol Genet

4510-27

23

(17):

20140901

PMID:24728190

DOI: 10.1093/hmg/ddu166

PMCID:

Downing NR, | Kim JI, | Williams JK, | Long JD, | Mills JA, | Paulsen JS, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

WHODAS 2.0 in prodromal Huntington disease: measures of functioning in neuropsychiatric disease

Eur J Hum Genet

958-63

22

(8):

20140801

PMID:24327189

DOI: 10.1038/ejhg.2013.275

PMCID: 4350592

Mielcarek M, | Inuabasi L, | Bondulich MK, | Muller T, | Osborne GF, | Franklin SA, | Smith DL, | Neueder A, | Rosinski J, | Rattray I, | Protti A, | Bates GP |

Dysfunction of the CNS-heart axis in mouse models of Huntington's disease

PLoS Genet

e1004550

10

(8):

20140801

PMID:25101683

DOI: 10.1371/journal.pgen.1004550

PMCID: 4125112

Dominguez C, | Munoz-Sanjuan I |

Foundation-directed therapeutic development in Huntington's disease

J Med Chem

5479-88

57

(13):

20140710

PMID:24432836

DOI: 10.1021/jm4009295

PMCID:

Plotkin JL, | Day M, | Peterson JD, | Xie Z, | Kress GJ, | Rafalovich I, | Kondapalli J, | Gertler TS, | Flajolet M, | Greengard P, | Stavarache M, | Kaplitt MG, | Rosinski J, | Chan CS, | Surmeier DJ |

Impaired TrkB receptor signaling underlies corticostriatal dysfunction in Huntington's disease

Neuron

178-88

83

(1):

20140702

PMID:24991961

DOI: 10.1016/j.neuron.2014.05.032

PMCID: 4131293

Sepers MD, | Raymond LA |

Mechanisms of synaptic dysfunction and excitotoxicity in Huntington's disease

Drug Discov Today

990-6

19

(7):

20140701

PMID:24603212

DOI: 10.1016/j.drudis.2014.02.006

PMCID:

Signer, E |

Through postdoc eyes

Res Microbiol

346-7

165

(5):

20140601

PMID:24859142

DOI: 10.1016/j.resmic.2014.05.029

PMCID:

Ross CA, | Aylward EH, | Wild EJ, | Langbehn DR, | Long JD, | Warner JH, | Scahill RI, | Leavitt BR, | Stout JC, | Paulsen JS, | Reilmann R, | Unschuld PG, | Wexler A, | Margolis RL, | Tabrizi SJ |

Huntington disease: natural history, biomarkers and prospects for therapeutics

Nat Rev Neurol

204-16

10

(4):

20140401

PMID:24614516

DOI: 10.1038/nrneurol.2014.24

PMCID:

Beglinger LJ, | Adams WH, | Langbehn D, | Fiedorowicz JG, | Jorge R, | Biglan K, | Caviness J, | Olson B, | Robinson RG, | Kieburtz K, | Paulsen JS |

Results of the citalopram to enhance cognition in Huntington disease trial

Mov Disord

401-5

29

(3):

20140301

PMID:24375941

DOI: 10.1002/mds.25750

PMCID: 3960314

Dodel R, | Jonsson B, | Reese JP, | Winter Y, | Martinez-Martin P, | Holloway R, | Sampaio C, | Ruzicka E, | Hawthorne G, | Oertel W, | Poewe W, | Stebbins G, | Rascol O, | Goetz CG, | Schrag A |

Measurement of costs and scales for outcome evaluation in health economic studies of Parkinson's disease

Mov Disord

169-76

29

(2):

20140201

PMID:23861335

DOI: 10.1002/mds.25571

PMCID:

Bard J, | Wall MD, | Lazari O, | Arjomand J, | Munoz-Sanjuan I |

Advances in huntington disease drug discovery: novel approaches to model disease phenotypes

J Biomol Screen

191-204

19

(2):

20140201

PMID:24196395

DOI: 10.1177/1087057113510320

PMCID:

Paulsen JS, | Long JD, | Johnson HJ, | Aylward EH, | Ross CA, | Williams JK, | Nance MA, | Erwin CJ, | Westervelt HJ, | Harrington DL, | Bockholt HJ, | Zhang Y, | McCusker EA, | Chiu EM, | Panegyres PK, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

Clinical and biomarker changes in premanifest Huntington disease show trial feasibility: A decade of the PREDICT-HD study

Front Aging Neurosci

78

6

():

20140101

PMID:24795630

DOI: 10.3389/fnagi.2014.00078

PMCID: 4000999

Vodicka P, | Lim J, | Williams DT, | Kegel KB, | Chase K, | Park H, | Marchionini D, | Wilkinson S, | Mead T, | Birch H, | Yates D, | Lyons K, | Dominguez C, | Beconi M, | Yue Z, | Aronin N, | DiFiglia M |

Assessment of chloroquine treatment for modulating autophagy flux in brain of WT and HD mice

J Huntingtons Dis

159-74

3

(2):

20140101

PMID:25062859

DOI: 10.3233/JHD-130081

PMCID:

Fodale V, | Kegulian NC, | Verani M, | Cariulo C, | Azzollini L, | Petricca L, | Daldin M, | Boggio R, | Padova A, | Kuhn R, | Pacifici R, | Macdonald D, | Schoenfeld RC, | Park H, | Isas JM, | Langen R, | Weiss A, | Caricasole A |

Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy

PLoS One

e112262

9

(12):

20140101

PMID:25464275

DOI: 10.1371/journal.pone.0112262

PMCID: 4251833

Todd D, | Gowers I, | Dowler SJ, | Wall MD, | McAllister G, | Fischer DF, | Dijkstra S, | Fratantoni SA, | van de Bospoort R, | Veenman-Koepke J, | Flynn G, | Arjomand J, | Dominguez C, | Munoz-Sanjuan I, | Wityak J, | Bard JA |

A monoclonal antibody TrkB receptor agonist as a potential therapeutic for Huntington's disease

PLoS One

e87923

9

(2):

20140101

PMID:24503862

DOI: 10.1371/journal.pone.0087923

PMCID: 3913682

Sutcliffe JS, | Beaumont V, | Watson JM, | Chew CS, | Beconi M, | Hutcheson DM, | Dominguez C, | Munoz-Sanjuan I |

Efficacy of selective PDE4D negative allosteric modulators in the object retrieval task in female cynomolgus monkeys (Macaca fascicularis)

PLoS One

e102449

9

(7):

20140101

PMID:25050979

DOI: 10.1371/journal.pone.0102449

PMCID: 4106781

Cachope R, | Cheer JF |

Local control of striatal dopamine release

Front Behav Neurosci

188

8

():

20140101

PMID:24904339

DOI: 10.3389/fnbeh.2014.00188

PMCID: 4033078

Beglinger LJ, | Adams WH, | Langbehn D, | Fiedorowicz JG, | Caviness J, | Biglan K, | Olson B, | Paulsen JS |

Does interval between screening and baseline matter in HD cognitive clinical trials?

J Huntingtons Dis

139-44

3

(2):

20140101

PMID:25062857

DOI: 10.3233/JHD-140100

PMCID:

Beaumont V, | Park L, | Rassoulpour A, | Dijkman U, | Heikkinen T, | Lehtimaki K, | Kontkanen O, | Al Nackkash R, | Bates GP, | Gleyzes M, | Steidl E, | Ramboz S, | Murphy C, | Beconi MG, | Dominguez C, | Munoz-Sanjuan I |

The PDE1/5 inhibitor SCH-51866 does not modify disease progression in the R6/2 mouse model of Huntington's Disease

PLoS Curr

6

():

20140101

PMID:24558637

DOI: 10.1371/currents.hd.3304e87e460b4bb0dc519a29f4deccca

PMCID: 3923778

Anderson KE, | Divino V, | DeKoven M, | Langbehn D, | Warner JH, | Giuliano J, | Lee WC |

Interventional differences among Huntington's disease patients by disease progression in commercial and medicaid populations

J Huntingtons Dis

355-63

3

(4):

20140101

PMID:25575957

DOI: 10.3233/JHD-140124

PMCID:

Menalled LB, | Kudwa AE, | Oakeshott S, | Farrar A, | Paterson N, | Filippov I, | Miller S, | Kwan M, | Olsen M, | Beltran J, | Torello J, | Fitzpatrick J, | Mushlin R, | Cox K, | McConnell K, | Mazzella M, | He D, | Osborne GF, | Al-Nackkash R, | Bates GP, | Tuunanen P, | Lehtimaki K, | Brunner D, | Ghavami A, | Ramboz S, | Park L, | Macdonald D, | Munoz-Sanjuan I, | Howland D |

Genetic deletion of transglutaminase 2 does not rescue the phenotypic deficits observed in R6/2 and zQ175 mouse models of Huntington's disease

PLoS One

e99520

9

(6):

20140101

PMID:24955833

DOI: 10.1371/journal.pone.0099520

PMCID: 4067284

Macdonald D, | Tessari MA, | Boogaard I, | Smith M, | Pulli K, | Szynol A, | Albertus F, | Lamers MB, | Dijkstra S, | Kordt D, | Reindl W, | Herrmann F, | McAllister G, | Fischer DF, | Munoz-Sanjuan I |

Quantification assays for total and polyglutamine-expanded huntingtin proteins

PLoS One

e96854

9

(5):

20140101

PMID:24816435

DOI: 10.1371/journal.pone.0096854

PMCID: 4016121

2013

Burli RW, | Luckhurst CA, | Aziz O, | Matthews KL, | Yates D, | Lyons KA, | Beconi M, | McAllister G, | Breccia P, | Stott AJ, | Penrose SD, | Wall M, | Lamers M, | Leonard P, | Muller I, | Richardson CM, | Jarvis R, | Stones L, | Hughes S, | Wishart G, | Haughan AF, | O`Connell C, | Mead T, | McNeil H, | Vann J, | Mangette J, | Maillard M, | Beaumont V, | Munoz-Sanjuan I, | Dominguez C |

Design, synthesis, and biological evaluation of potent and selective class IIa histone deacetylase (HDAC) inhibitors as a potential therapy for Huntington's disease

J Med Chem

9934-54

56

(24):

20131227

PMID:24261862

DOI: 10.1021/jm4011884

PMCID:

Deng YP, | Wong T, | Bricker-Anthony C, | Deng B, | Reiner A |

Loss of corticostriatal and thalamostriatal synaptic terminals precedes striatal projection neuron pathology in heterozygous Q140 Huntington's disease mice

Neurobiol Dis

89-107

60

():

20131201

PMID:23969239

DOI: 10.1016/j.nbd.2013.08.009

PMCID: 3808190

Mielcarek M, | Landles C, | Weiss A, | Bradaia A, | Seredenina T, | Inuabasi L, | Osborne GF, | Wadel K, | Touller C, | Butler R, | Robertson J, | Franklin SA, | Smith DL, | Park L, | Marks PA, | Wanker EE, | Olson EN, | Luthi-Carter R, | van der Putten H, | Beaumont V, | Bates GP |

HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration

PLoS Biol

e1001717

11

(11):

20131101

PMID:24302884

DOI: 10.1371/journal.pbio.1001717

PMCID: 3841096

Elble R, | Bain P, | Forjaz MJ, | Haubenberger D, | Testa C, | Goetz CG, | Leentjens AF, | Martinez-Martin P, | Pavy-Le Traon A, | Post B, | Sampaio C, | Stebbins GT, | Weintraub D, | Schrag A |

Task force report: scales for screening and evaluating tremor: critique and recommendations

Mov Disord

1793-800

28

(13):

20131101

PMID:24038576

DOI: 10.1002/mds.25648

PMCID:

Epping EA, | Mills JA, | Beglinger LJ, | Fiedorowicz JG, | Craufurd D, | Smith MM, | Groves M, | Bijanki KR, | Downing N, | Williams JK, | Long JD, | Paulsen JS, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

Characterization of depression in prodromal Huntington disease in the neurobiological predictors of HD (PREDICT-HD) study

J Psychiatr Res

1423-31

47

(10):

20131001

PMID:23790259

DOI: 10.1016/j.jpsychires.2013.05.026

PMCID: 3808084

Georgiou-Karistianis N, | Poudel GR, | Dominguez DJf, | Langmaid R, | Gray MA, | Churchyard A, | Chua P, | Borowsky B, | Egan GF, | Stout JC |

Functional and connectivity changes during working memory in Huntington's disease: 18 month longitudinal data from the IMAGE-HD study

Brain Cogn

80-91

83

(1):

20131001

PMID:23938592

DOI: 10.1016/j.bandc.2013.07.004

PMCID:

Maillard MC, | Dominguez C, | Gemkow MJ, | Krieger F, | Park H, | Schaertl S, | Winkler D, | Munoz-Sanjuan I |

A label-free LC/MS/MS-based enzymatic activity assay for the detection of genuine caspase inhibitors and SAR development

J Biomol Screen

868-78

18

(8):

20130901

PMID:23796689

DOI: 10.1177/1087057113492851

PMCID:

Delmaire C, | Dumas EM, | Sharman MA, | van den Bogaard SJ, | Valabregue R, | Jauffret C, | Justo D, | Reilmann R, | Stout JC, | Craufurd D, | Tabrizi SJ, | Roos RA, | Durr A, | Lehericy S |

The structural correlates of functional deficits in early huntington's disease

Hum Brain Mapp

2141-53

34

(9):

20130901

PMID:22438242

DOI: 10.1002/hbm.22055

PMCID:

Winkler D, | Beconi M, | Toledo-Sherman LM, | Prime M, | Ebneth A, | Dominguez C, | Munoz-Sanjuan I |

Development of LC/MS/MS, high-throughput enzymatic and cellular assays for the characterization of compounds that inhibit kynurenine monooxygenase (KMO)

J Biomol Screen

879-89

18

(8):

20130901

PMID:23690293

DOI: 10.1177/1087057113489731

PMCID:

Lee JM, | Galkina EI, | Levantovsky RM, | Fossale E, | Anne Anderson M, | Gillis T, | Srinidhi Mysore J, | Coser KR, | Shioda T, | Zhang B, | Furia MD, | Derry J, | Kohane IS, | Seong IS, | Wheeler VC, | Gusella JF, | MacDonald ME |

Dominant effects of the Huntington's disease HTT CAG repeat length are captured in gene-expression data sets by a continuous analysis mathematical modeling strategy

Hum Mol Genet

3227-38

22

(16):

20130815

PMID:23595883

DOI: 10.1093/hmg/ddt176

PMCID: 3723309

Bonner-Jackson A, | Long JD, | Westervelt H, | Tremont G, | Aylward E, | Paulsen JS, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

Cognitive reserve and brain reserve in prodromal Huntington's disease

J Int Neuropsychol Soc

739-50

19

(7):

20130801

PMID:23702309

DOI: 10.1017/S1355617713000507

PMCID: 3720793

Divino V, | Dekoven M, | Warner JH, | Giuliano J, | Anderson KE, | Langbehn D, | Lee WC |

The direct medical costs of Huntington's disease by stage. A retrospective commercial and Medicaid claims data analysis

J Med Econ

1043-50

16

(8):

20130801

PMID:23789925

DOI: 10.3111/13696998.2013.818545

PMCID:

Delli Carri A, | Onorati M, | Castiglioni V, | Faedo A, | Camnasio S, | Toselli M, | Biella G, | Cattaneo E |

Human pluripotent stem cell differentiation into authentic striatal projection neurons

Stem Cell Rev

461-74

9

(4):

20130801

PMID:23625190

DOI: 10.1007/s12015-013-9441-8

PMCID:

Vellas B, | Carrillo MC, | Sampaio C, | Brashear HR, | Siemers E, | Hampel H, | Schneider LS, | Weiner M, | Doody R, | Khachaturian Z, | Cedarbaum J, | Grundman M, | Broich K, | Giacobini E, | Dubois B, | Sperling R, | Wilcock GK, | Fox N, | Scheltens P, | Touchon J, | Hendrix S, | Andrieu S, | Aisen P, | EU US CTAD Task Force Members |

Designing drug trials for Alzheimer's disease: what we have learned from the release of the phase III antibody trials: a report from the EU/US/CTAD Task Force

Alzheimers Dement

438-44

9

(4):

20130701

PMID:23809364

DOI: 10.1016/j.jalz.2013.03.007

PMCID:

Tabrizi SJ, | Scahill RI, | Owen G, | Durr A, | Leavitt BR, | Roos RA, | Borowsky B, | Landwehrmeyer B, | Frost C, | Johnson H, | Craufurd D, | Reilmann R, | Stout JC, | Langbehn DR, | Track-Hd Investigators |

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data

Lancet Neurol

637-49

12

(7):

20130701

PMID:23664844

DOI: 10.1016/S1474-4422(13)70088-7

PMCID:

Borowsky B, | Warner J, | Leavitt BR, | Tabrizi SJ, | Roos RA, | Durr A, | Becker C, | Sampaio C, | Tobin AJ, | Schulman H |

8OHdG is not a biomarker for Huntington disease state or progression

Neurology

1934-41

80

(21):

20130521

PMID:23616162

DOI: 10.1212/WNL.0b013e318293e1a1

PMCID: 3716347

Labuschagne I, | Jones R, | Callaghan J, | Whitehead D, | Dumas EM, | Say MJ, | Hart EP, | Justo D, | Coleman A, | Dar Santos RC, | Frost C, | Craufurd D, | Tabrizi SJ, | Stout JC, | Track-Hd Investigators |

Emotional face recognition deficits and medication effects in pre-manifest through stage-II Huntington's disease

Psychiatry Res

118-26

207

(1-2):

20130515

PMID:23051887

DOI: 10.1016/j.psychres.2012.09.022

PMCID:

Georgiou-Karistianis N, | Gray MA, | Dominguez DJf, | Dymowski AR, | Bohanna I, | Johnston LA, | Churchyard A, | Chua P, | Stout JC, | Egan GF |

Automated differentiation of pre-diagnosis Huntington's disease from healthy control individuals based on quadratic discriminant analysis of the basal ganglia: the IMAGE-HD study

Neurobiol Dis

82-92

51

():

20130301

PMID:23069680

DOI: 10.1016/j.nbd.2012.10.001

PMCID:

Papp KV, | Snyder PJ, | Mills JA, | Duff K, | Westervelt HJ, | Long JD, | Lourens S, | Paulsen JS |

Measuring executive dysfunction longitudinally and in relation to genetic burden, brain volumetrics, and depression in prodromal Huntington disease

Arch Clin Neuropsychol

156-68

28

(2):

20130301

PMID:23246934

DOI: 10.1093/arclin/acs105

PMCID: 3569950

Sathasivam K, | Neueder A, | Gipson TA, | Landles C, | Benjamin AC, | Bondulich MK, | Smith DL, | Faull RL, | Roos RA, | Howland D, | Detloff PJ, | Housman DE, | Bates GP |

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

Proc Natl Acad Sci U S A

2366-70

110

(6):

20130205

PMID:23341618

DOI: 10.1073/pnas.1221891110

PMCID: 3568346

van den Bogaard SJ, | Dumas EM, | Hart EP, | Milles J, | Reilmann R, | Stout JC, | Craufurd D, | Gibbard CR, | Tabrizi SJ, | van Buchem MA, | van der Grond J, | Roos RA |

Magnetization transfer imaging in premanifest and manifest huntington disease: a 2-year follow-up

AJNR Am J Neuroradiol

317-22

34

(2):

20130201

PMID:22918430

DOI: 10.3174/ajnr.A3303

PMCID:

Oakeshott S, | Farrar A, | Port R, | Cummins-Sutphen J, | Berger J, | Watson-Johnson J, | Ramboz S, | Howland D, | Brunner D |

Deficits in a simple visual go/no-go discrimination task in two mouse models of Huntington's Disease

PLoS Curr

5

():

20130101

PMID:24270512

DOI: 10.1371/currents.hd.fe74c94bdd446a0470f6f905a30b5dd1

PMCID: 3828223

Morton AJ, | Howland DS |

Large genetic animal models of Huntington's Disease

J Huntingtons Dis

3-19

2

(1):

20130101

PMID:25063426

DOI: 10.3233/JHD-130050

PMCID:

Gray MA, | Egan GF, | Ando A, | Churchyard A, | Chua P, | Stout JC, | Georgiou-Karistianis N |

Prefrontal activity in Huntington's disease reflects cognitive and neuropsychiatric disturbances: the IMAGE-HD study

Exp Neurol

218-28

239

():

20130101

PMID:23123406

DOI: 10.1016/j.expneurol.2012.10.020

PMCID:

Liu W, | Chaurette J, | Pfister EL, | Kennington LA, | Chase KO, | Bullock J, | Vonsattel JP, | Faull RL, | Macdonald D, | DiFiglia M, | Zamore PD, | Aronin N |

Increased steady-state mutant huntingtin mRNA in Huntington's Disease brain

J Huntingtons Dis

491-500

2

(4):

20130101

PMID:25062733

DOI: 10.3233/JHD-130079

PMCID:

Kudwa AE, | Menalled LB, | Oakeshott S, | Murphy C, | Mushlin R, | Fitzpatrick J, | Miller SF, | McConnell K, | Port R, | Torello J, | Howland D, | Ramboz S, | Brunner D |

Increased body weight of the BAC HD transgenic mouse model of Huntington's Disease accounts for some but not all of the observed HD-like motor deficits

PLoS Curr

5

():

20130101

PMID:24042107

DOI: 10.1371/currents.hd.0ab4f3645aff523c56ecc8ccbe41a198

PMCID: 3770835

Read J, | Jones R, | Owen G, | Leavitt BR, | Coleman A, | Roos RA, | Dumas EM, | Durr A, | Justo D, | Say M, | Stout JC, | Tabrizi SJ, | Craufurd D, | Track-Hd investigators |

Quality of life in Huntington's disease: a comparative study investigating the impact for those with pre-manifest and early manifest disease, and their partners

J Huntingtons Dis

159-75

2

(2):

20130101

PMID:25063513

DOI: 10.3233/JHD-130051

PMCID:

Valencia A, | Sapp E, | Kimm JS, | McClory H, | Ansong KA, | Yohrling G, | Kwak S, | Kegel KB, | Green KM, | Shaffer SA, | Aronin N, | DiFiglia M |

Striatal synaptosomes from Hdh140Q/140Q knock-in mice have altered protein levels, novel sites of methionine oxidation, and excess glutamate release after stimulation

J Huntingtons Dis

459-75

2

(4):

20130101

PMID:24696705

DOI: 10.3233/JHD-130080

PMCID: 3970728

Baxa M, | Hruska-Plochan M, | Juhas S, | Vodicka P, | Pavlok A, | Juhasova J, | Miyanohara A, | Nejime T, | Klima J, | Macakova M, | Marsala S, | Weiss A, | Kubickova S, | Musilova P, | Vrtel R, | Sontag EM, | Thompson LM, | Schier J, | Hansikova H, | Howland DS, | Cattaneo E, | DiFiglia M, | Marsala M, | Motlik J |

A transgenic minipig model of Huntington's Disease

J Huntingtons Dis

47-68

2

(1):

20130101

PMID:25063429

DOI: 10.3233/JHD-130001

PMCID:

Balci F, | Oakeshott S, | Shamy JL, | El-Khodor BF, | Filippov I, | Mushlin R, | Port R, | Connor D, | Paintdakhi A, | Menalled L, | Ramboz S, | Howland D, | Kwak S, | Brunner D |

High-throughput automated phenotyping of two genetic mouse models of Huntington's Disease

PLoS Curr

5

():

20130101

PMID:23863947

DOI: 10.1371/currents.hd.124aa0d16753f88215776fba102ceb29

PMCID: 3710674

2012

Beconi MG, | Yates D, | Lyons K, | Matthews K, | Clifton S, | Mead T, | Prime M, | Winkler D, | O`Connell C, | Walter D, | Toledo-Sherman L, | Munoz-Sanjuan I, | Dominguez C |

Metabolism and pharmacokinetics of JM6 in mice: JM6 is not a prodrug for Ro-61-8048

Drug Metab Dispos

2297-306

40

(12):

20121201

PMID:22942319

DOI: 10.1124/dmd.112.046532

PMCID:

Weiss A, | Trager U, | Wild EJ, | Grueninger S, | Farmer R, | Landles C, | Scahill RI, | Lahiri N, | Haider S, | Macdonald D, | Frost C, | Bates GP, | Bilbe G, | Kuhn R, | Andre R, | Tabrizi SJ |

Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression

J Clin Invest

3731-6

122

(10):

20121001

PMID:22996692

DOI: 10.1172/JCI64565

PMCID: 3461928

Reiner A, | Wang HB, | Del Mar N, | Sakata K, | Yoo W, | Deng YP |

BDNF may play a differential role in the protective effect of the mGluR2/3 agonist LY379268 on striatal projection neurons in R6/2 Huntington's disease mice

Brain Res

161-72

1473

():

20120914

PMID:22820300

DOI: 10.1016/j.brainres.2012.07.026

PMCID: 3428435

Prime ME, | Brookfield FA, | Courtney SM, | Gaines S, | Marston RW, | Ichihara O, | Li M, | Vaidya D, | Williams H, | Pedret-Dunn A, | Reed L, | Schaertl S, | Toledo-Sherman L, | Beconi M, | Macdonald D, | Munoz-Sanjuan I, | Dominguez C, | Wityak J |

Irreversible 4-Aminopiperidine Transglutaminase 2 Inhibitors for Huntington's Disease

ACS Med Chem Lett

731-5

3

(9):

20120913

PMID:24900540

DOI: 10.1021/ml3001352

PMCID: 4025765

Wolf RC, | Sambataro F, | Vasic N, | Wolf ND, | Thomann PA, | Saft C, | Landwehrmeyer GB, | Orth M |

Default-mode network changes in preclinical Huntington's disease

Exp Neurol

191-8

237

(1):

20120901

PMID:22742947

DOI: 10.1016/j.expneurol.2012.06.014

PMCID:

Scahill RI, | Wild EJ, | Tabrizi SJ |

Biomarkers for Huntington's disease: an update

Expert Opin Med Diagn

371-5

6

(5):

20120901

PMID:23480802

DOI: 10.1517/17530059.2012.701205

PMCID:

HD iPSC Consortium |

Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes

Cell Stem Cell

264-78

11

(2):

20120803

PMID:22748968

DOI: 10.1016/j.stem.2012.04.027

PMCID: 3804072

Dumas EM, | Versluis MJ, | van den Bogaard SJ, | van Osch MJ, | Hart EP, | van Roon-Mom WM, | van Buchem MA, | Webb AG, | van der Grond J, | Roos RA, | Track-Hd investigators |

Elevated brain iron is independent from atrophy in Huntington's Disease

Neuroimage

558-64

61

(3):

20120702

PMID:22480728

DOI: 10.1016/j.neuroimage.2012.03.056

PMCID:

Reiner A, | Lafferty DC, | Wang HB, | Del Mar N, | Deng YP |

The group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease mice

Neurobiol Dis

75-91

47

(1):

20120701

PMID:22472187

DOI: 10.1016/j.nbd.2012.03.025

PMCID: 3376646

Kordasiewicz HB, | Stanek LM, | Wancewicz EV, | Mazur C, | McAlonis MM, | Pytel KA, | Artates JW, | Weiss A, | Cheng SH, | Shihabuddin LS, | Hung G, | Bennett CF, | Cleveland DW |

Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis

Neuron

1031-44

74

(6):

20120621

PMID:22726834

DOI: 10.1016/j.neuron.2012.05.009

PMCID: 3383626

Long JD, | Matson WR, | Juhl AR, | Leavitt BR, | Paulsen JS, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

8OHdG as a marker for Huntington disease progression

Neurobiol Dis

625-34

46

(3):

20120601

PMID:22414782

DOI: 10.1016/j.nbd.2012.02.012

PMCID: 3784019

Brooks SP, | Janghra N, | Workman VL, | Bayram-Weston Z, | Jones L, | Dunnett SB |

Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice

Brain Res Bull

94-103

88

(2-3):

20120601

PMID:21272613

DOI: 10.1016/j.brainresbull.2011.01.010

PMCID:

Gafni J, | Papanikolaou T, | Degiacomo F, | Holcomb J, | Chen S, | Menalled L, | Kudwa A, | Fitzpatrick J, | Miller S, | Ramboz S, | Tuunanen PI, | Lehtimaki KK, | Yang XW, | Park L, | Kwak S, | Howland D, | Park H, | Ellerby LM |

Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment

J Neurosci

7454-65

32

(22):

20120530

PMID:22649225

DOI: 10.1523/JNEUROSCI.6379-11.2012

PMCID: 3454486

Nucifora LG, | Burke KA, | Feng X, | Arbez N, | Zhu S, | Miller J, | Yang G, | Ratovitski T, | Delannoy M, | Muchowski PJ, | Finkbeiner S, | Legleiter J, | Ross CA, | Poirier MA |

Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein

J Biol Chem

16017-28

287

(19):

20120504

PMID:22433867

DOI: 10.1074/jbc.M111.252577

PMCID: 3346083

Uribe V, | Wong BK, | Graham RK, | Cusack CL, | Skotte NH, | Pouladi MA, | Xie Y, | Feinberg K, | Ou Y, | Ouyang Y, | Deng Y, | Franciosi S, | Bissada N, | Spreeuw A, | Zhang W, | Ehrnhoefer DE, | Vaid K, | Miller FD, | Deshmukh M, | Howland D, | Hayden MR |

Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice

Hum Mol Genet

1954-67

21

(9):

20120501

PMID:22262731

DOI: 10.1093/hmg/dds005

PMCID: 3315204

Rossin F, | D`Eletto M, | Macdonald D, | Farrace MG, | Piacentini M |

TG2 transamidating activity acts as a reostat controlling the interplay between apoptosis and autophagy

Amino Acids

1793-802

42

(5):

20120501

PMID:21479826

DOI: 10.1007/s00726-011-0899-x

PMCID:

Aylward EH, | Liu D, | Nopoulos PC, | Ross CA, | Pierson RK, | Mills JA, | Long JD, | Paulsen JS, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

Striatal volume contributes to the prediction of onset of Huntington disease in incident cases

Biol Psychiatry

822-8

71

(9):

20120501

PMID:21907324

DOI: 10.1016/j.biopsych.2011.07.030

PMCID: 3237730

Rowe KC, | Paulsen JS, | Langbehn DR, | Wang C, | Mills J, | Beglinger LJ, | Smith MM, | Epping EA, | Fiedorowicz JG, | Duff K, | Ruggle A, | Moser DJ, | Predict-Hd Investigators of the Huntington Study Group |

Patterns of serotonergic antidepressant usage in prodromal Huntington disease

Psychiatry Res

309-14

196

(2-3):

20120430

PMID:22397915

DOI: 10.1016/j.psychres.2011.09.005

PMCID: 3763706

Vellas B, | Hampel H, | Rouge-Bugat ME, | Grundman M, | Andrieu S, | Abu-Shakra S, | Bateman R, | Berman R, | Black R, | Carrillo M, | Donohue M, | Mintun M, | Morris J, | Petersen R, | Thomas RG, | Suhy J, | Schneider L, | Seely L, | Tariot P, | Touchon J, | Weiner M, | Sampaio C, | Aisen P, | Task Force Participants |

Alzheimer's disease therapeutic trials: EU/US Task Force report on recruitment, retention, and methodology

J Nutr Health Aging

339-45

16

(4):

20120401

PMID:22499454

DOI:

PMCID:

Beconi MG, | Howland D, | Park L, | Lyons K, | Giuliano J, | Dominguez C, | Munoz-Sanjuan I, | Pacifici R |

Pharmacokinetics of memantine in rats and mice

PLoS Curr

RRN1291

3

():

20120215

PMID:22307216

DOI: 10.1371/currents.RRN1291

PMCID: 3269340

Prime ME, | Andersen OA, | Barker JJ, | Brooks MA, | Cheng RK, | Toogood-Johnson I, | Courtney SM, | Brookfield FA, | Yarnold CJ, | Marston RW, | Johnson PD, | Johnsen SF, | Palfrey JJ, | Vaidya D, | Erfan S, | Ichihara O, | Felicetti B, | Palan S, | Pedret-Dunn A, | Schaertl S, | Sternberger I, | Ebneth A, | Scheel A, | Winkler D, | Toledo-Sherman L, | Beconi M, | Macdonald D, | Munoz-Sanjuan I, | Dominguez C, | Wityak J |

Discovery and structure-activity relationship of potent and selective covalent inhibitors of transglutaminase 2 for Huntington's disease

J Med Chem

1021-46

55

(3):

20120209

PMID:22224594

DOI: 10.1021/jm201310y

PMCID:

Beconi M, | Aziz O, | Matthews K, | Moumne L, | O`Connell C, | Yates D, | Clifton S, | Pett H, | Vann J, | Crowley L, | Haughan AF, | Smith DL, | Woodman B, | Bates GP, | Brookfield F, | Burli RW, | McAllister G, | Dominguez C, | Munoz-Sanjuan I, | Beaumont V |

Oral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivo

PLoS One

e44498

7

(9):

20120101

PMID:22973455

DOI: 10.1371/journal.pone.0044498

PMCID: 3433414

Tabrizi SJ, | Reilmann R, | Roos RAC, | Durr A, | Leavitt B, | Owen G, | Jones R, | Johnson H, | Craufurd D, | Hicks SL, | Kennard C, | Landwehrmeyer B, | Stout JC, | Borowsky B, | Scahill RI, | Frost C, | Langbehn DR |

Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data

The Lancet Neurology

42-53

11

(1):

20120101

PMID:22137354

DOI: 10.1016/s1474-4422(11)70263-0

PMCID:

Galvan L, | Andre VM, | Wang EA, | Cepeda C, | Levine MS |

Functional differences between direct and indirect striatal output pathways in Huntington's disease

J Huntingtons Dis

17-25

1

(1):

20120101

PMID:25063187

DOI: 10.3233/JHD-2012-120009

PMCID: 4116084

Oakeshott S, | Port R, | Cummins-Sutphen J, | Berger J, | Watson-Johnson J, | Ramboz S, | Paterson N, | Kwak S, | Howland D, | Brunner D |

A mixed fixed ratio/progressive ratio procedure reveals an apathy phenotype in the BAC HD and the z_Q175 KI mouse models of Huntington's disease

PLoS Curr

e4f972cffe82c0

4

():

20120101

PMID:23925262

DOI: 10.1371/4f972cffe82c0

PMCID: 3729251

Menalled LB, | Kudwa AE, | Miller S, | Fitzpatrick J, | Watson-Johnson J, | Keating N, | Ruiz M, | Mushlin R, | Alosio W, | McConnell K, | Connor D, | Murphy C, | Oakeshott S, | Kwan M, | Beltran J, | Ghavami A, | Brunner D, | Park LC, | Ramboz S, | Howland D |

Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175

PLoS One

e49838

7

(12):

20120101

PMID:23284626

DOI: 10.1371/journal.pone.0049838

PMCID: 3527464

Heikkinen T, | Lehtimaki K, | Vartiainen N, | Puolivali J, | Hendricks SJ, | Glaser JR, | Bradaia A, | Wadel K, | Touller C, | Kontkanen O, | Yrjanheikki JM, | Buisson B, | Howland D, | Beaumont V, | Munoz-Sanjuan I, | Park LC |

Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington's disease

PLoS One

e50717

7

(12):

20120101

PMID:23284644

DOI: 10.1371/journal.pone.0050717

PMCID: 3527436

Quarrell OW, | Handley O, | O`Donovan K, | Dumoulin C, | Ramos-Arroyo M, | Biunno I, | Bauer P, | Kline M, | Landwehrmeyer GB, | European Huntington`s Disease Network |

Discrepancies in reporting the CAG repeat lengths for Huntington's disease

Eur J Hum Genet

20-6

20

(1):

20120101

PMID:21811303

DOI: 10.1038/ejhg.2011.136

PMCID: 3234505

2011

Maillard MC, | Brookfield FA, | Courtney SM, | Eustache FM, | Gemkow MJ, | Handel RK, | Johnson LC, | Johnson PD, | Kerry MA, | Krieger F, | Meniconi M, | Munoz-Sanjuan I, | Palfrey JJ, | Park H, | Schaertl S, | Taylor MG, | Weddell D, | Dominguez C |

Exploiting differences in caspase-2 and -3 S(2) subsites for selectivity: structure-based design, solid-phase synthesis and in vitro activity of novel substrate-based caspase-2 inhibitors

Bioorg Med Chem

5833-51

19

(19):

20111001

PMID:21903398

DOI: 10.1016/j.bmc.2011.08.020

PMCID:

Muller I, | Lamers MB, | Ritchie AJ, | Dominguez C, | Munoz-Sanjuan I, | Kiselyov A |

Structure of human caspase-6 in complex with Z-VAD-FMK: New peptide binding mode observed for the non-canonical caspase conformation

Bioorg Med Chem Lett

5244-7

21

(18):

20110915

PMID:21820899

DOI: 10.1016/j.bmcl.2011.07.041

PMCID:

Huang K, | Sanders SS, | Kang R, | Carroll JB, | Sutton L, | Wan J, | Singaraja R, | Young FB, | Liu L, | El-Husseini A, | Davis NG, | Hayden MR |

Wild-type HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14

Hum Mol Genet

3356-65

20

(17):

20110901

PMID:21636527

DOI: 10.1093/hmg/ddr242

PMCID: 3153302

van den Bogaard SJ, | Dumas EM, | Ferrarini L, | Milles J, | van Buchem MA, | van der Grond J, | Roos RA |

Shape analysis of subcortical nuclei in Huntington's disease, global versus local atrophy--results from the TRACK-HD study

J Neurol Sci

60-8

307

(1-2):

20110815

PMID:21624624

DOI: 10.1016/j.jns.2011.05.015

PMCID:

Hobbs NZ, | Pedrick AV, | Say MJ, | Frost C, | Dar Santos R, | Coleman A, | Sturrock A, | Craufurd D, | Stout JC, | Leavitt BR, | Barnes J, | Tabrizi SJ, | Scahill RI |

The structural involvement of the cingulate cortex in premanifest and early Huntington's disease

Mov Disord

1684-90

26

(9):

20110801

PMID:21557312

DOI: 10.1002/mds.23747

PMCID:

Muller I, | Lamers MB, | Ritchie AJ, | Park H, | Dominguez C, | Munoz-Sanjuan I, | Maillard M, | Kiselyov A |

A new apo-caspase-6 crystal form reveals the active conformation of the apoenzyme

J Mol Biol

307-15

410

(2):

20110708

PMID:21621544

DOI: 10.1016/j.jmb.2011.05.020

PMCID:

O`Rourke JJ, | Beglinger LJ, | Smith MM, | Mills J, | Moser DJ, | Rowe KC, | Langbehn DR, | Duff K, | Stout JC, | Harrington DL, | Carlozzi N, | Paulsen JS |

The Trail Making Test in prodromal Huntington disease: contributions of disease progression to test performance

J Clin Exp Neuropsychol

567-79

33

(5):

20110601

PMID:21302170

DOI: 10.1080/13803395.2010.541228

PMCID: 3159183

Vaccarino AL, | Anderson K, | Borowsky B, | Duff K, | Giuliano J, | Guttman M, | Ho AK, | Orth M, | Paulsen JS, | Sills T, | van Kammen DP, | Evans KR, | Predict HD and | Registry Investigators Coordinators |

An item response analysis of the motor and behavioral subscales of the unified Huntington's disease rating scale in huntington disease gene expansion carriers

Mov Disord

877-84

26

(5):

20110401

PMID:21370269

DOI: 10.1002/mds.23574

PMCID: 3157755

Aylward EH, | Nopoulos PC, | Ross CA, | Langbehn DR, | Pierson RK, | Mills JA, | Johnson HJ, | Magnotta VA, | Juhl AR, | Paulsen JS, | Predict-HD Investigators and | Coordinators of Huntington Study Group |

Longitudinal change in regional brain volumes in prodromal Huntington disease

J Neurol Neurosurg Psychiatry

405-10

82

(4):

20110401

PMID:20884680

DOI: 10.1136/jnnp.2010.208264

PMCID: 3105627

Munoz-Sanjuan I, | Bates GP |

The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease

J Clin Invest

476-83

121

(2):

20110201

PMID:21285520

DOI: 10.1172/JCI45364

PMCID: 3026740

Vaccarino AL, | Sills T, | Anderson KE, | Endicott J, | Giuliano J, | Guttman M, | Ho AK, | Kupchak P, | Paulsen JS, | Warner JH, | Williams J, | Evans K |

Assessment of day-to-day functioning in prodromal and early Huntington Disease

PLoS Curr

RRN1262

3

():

20110101

PMID:21927718

DOI: 10.1371/currents.RRN1262

PMCID: 3172089

Vaccarino AL, | Sills T, | Anderson KE, | Borowsky B, | Craufurd D, | Giuliano J, | Goodman L, | Guttman M, | Kupchak P, | Ho AK, | Paulsen JS, | Stout J, | van Kammen DP, | Evans K |

Assessment of cognitive symptoms in prodromal and early huntington disease

PLoS Curr

RRN1250

3

():

20110101

PMID:22120841

DOI: 10.1371/currents.RRN1250

PMCID: 3201666

Stout JC, | Paulsen JS, | Queller S, | Solomon AC, | Whitlock KB, | Campbell JC, | Carlozzi N, | Duff K, | Beglinger LJ, | Langbehn DR, | Johnson SA, | Biglan KM, | Aylward EH |

Neurocognitive signs in prodromal Huntington disease

Neuropsychology

1-14

25

(1):

20110101

PMID:20919768

DOI: 10.1037/a0020937

PMCID: 3017660

Vaccarino AL, | Sills T, | Anderson KE, | Bachoud-Levi AC, | Borowsky B, | Craufurd D, | Duff K, | Giuliano J, | Groves M, | Guttman M, | Kupchak P, | Ho AK, | Paulsen JS, | Pedersen KF, | van Duijn E, | van Kammen DP, | Evans K |

Assessment of depression, anxiety and apathy in prodromal and early huntington disease

PLoS Curr

RRN1242

3

():

20110101

PMID:21731882

DOI: 10.1371/currents.RRN1242

PMCID: 3124013

Say MJ, | Jones R, | Scahill RI, | Dumas EM, | Coleman A, | Santos RC, | Justo D, | Campbell JC, | Queller S, | Shores EA, | Tabrizi SJ, | Stout JC, | Track-Hd Investigators |

Visuomotor integration deficits precede clinical onset in Huntington's disease

Neuropsychologia

264-70

49

(2):

20110101

PMID:21094653

DOI: 10.1016/j.neuropsychologia.2010.11.016

PMCID:

Vaccarino AL, | Anonymous, | Anderson KE, | Borowsky B, | Coccaro E, | Craufurd D, | Endicott J, | Giuliano J, | Groves M, | Guttman M, | Ho AK, | Kupchak P, | Paulsen JS, | Stanford MS, | van Kammen DP, | Watson D, | Wu KD, | Evans K |

Assessing behavioural manifestations prior to clinical diagnosis of huntington disease: "anger and irritability" and "obsessions and compulsions"

PLoS Curr

RRN1241

3

():

20110101

PMID:21826116

DOI: 10.1371/currents.RRN1241

PMCID: 3122583

Nopoulos PC, | Aylward EH, | Ross CA, | Mills JA, | Langbehn DR, | Johnson HJ, | Magnotta VA, | Pierson RK, | Beglinger LJ, | Nance MA, | Barker RA, | Paulsen JS, | Predict-Hd Investigators, | Coordinators of the Huntington Study Group |

Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment

Brain

137-42

134

(Pt 1):

20110101

PMID:20923788

DOI: 10.1093/brain/awq280

PMCID: 3025719

Oakeshott S, | Port RG, | Cummins-Sutphen J, | Watson-Johnson J, | Ramboz S, | Park L, | Howland D, | Brunner D |

HD mouse models reveal clear deficits in learning to perform a simple instrumental response

PLoS Curr

RRN1282

3

():

20110101

PMID:22512000

DOI: 10.1371/currents.RRN1282

PMCID: 3327146

Oakeshott S, | Balci F, | Filippov I, | Murphy C, | Port R, | Connor D, | Paintdakhi A, | Lesauter J, | Menalled L, | Ramboz S, | Kwak S, | Howland D, | Silver R, | Brunner D |

Circadian abnormalities in motor activity in a BAC transgenic mouse model of Huntington's Disease

PLoS Curr

RRN1225

3

():

20110101

PMID:21479110

DOI: 10.1371/currents.RRN1225

PMCID: 3072044

Fiedorowicz JG, | Mills JA, | Ruggle A, | Langbehn D, | Paulsen JS, | Predict-Hd Investigators of the Huntington Study Group |

Suicidal behavior in prodromal Huntington disease

Neurodegener Dis

483-90

8

(6):

20110101

PMID:21659725

DOI: 10.1159/000327754

PMCID: 3186721

Aylward E, | Mills J, | Liu D, | Nopoulos P, | Ross CA, | Pierson R, | Paulsen JS |

Association between Age and Striatal Volume Stratified by CAG Repeat Length in Prodromal Huntington Disease

PLoS Curr

RRN1235

3

():

20110101

PMID:21593963

DOI: 10.1371/currents.RRN1235

PMCID: 3092625

Vaccarino AL, | Sills T, | Anderson KE, | Biglan K, | Borowsky B, | Giuliano J, | Guttman M, | Ho AK, | Kennard C, | Kupchak P, | Landwehrmeyer GB, | Michell A, | Paulsen JS, | Reilmann R, | van Kammen DP, | Warner JH, | Evans K |

Assessment of motor symptoms and functional impact in prodromal and early huntington disease

PLoS Curr

RRN1244

2

():

20110101

PMID:21804956

DOI: 10.1371/currents.RRN1244

PMCID: 3114647

2010

Soneson C, | Fontes M, | Zhou Y, | Denisov V, | Paulsen JS, | Kirik D, | Petersen A, | Huntington Study Group Predict-HD Investigators |

Early changes in the hypothalamic region in prodromal Huntington disease revealed by MRI analysis

Neurobiol Dis

531-43

40

(3):

20101201

PMID:20682340

DOI: 10.1016/j.nbd.2010.07.013

PMCID: 2955781

Nopoulos PC, | Aylward EH, | Ross CA, | Johnson HJ, | Magnotta VA, | Juhl AR, | Pierson RK, | Mills J, | Langbehn DR, | Paulsen JS, | Predict-Hd Investigators Coordinators of Huntington Study Group |

Cerebral cortex structure in prodromal Huntington disease

Neurobiol Dis

544-54

40

(3):

20101201

PMID:20688164

DOI: 10.1016/j.nbd.2010.07.014

PMCID: 2955824

Leyva MJ, | Degiacomo F, | Kaltenbach LS, | Holcomb J, | Zhang N, | Gafni J, | Park H, | Lo DC, | Salvesen GS, | Ellerby LM, | Ellman JA |

Identification and evaluation of small molecule pan-caspase inhibitors in Huntington's disease models

Chem Biol

1189-200

17

(11):

20101124

PMID:21095569

DOI: 10.1016/j.chembiol.2010.08.014

PMCID: 3035168

Paulsen JS, | Wang C, | Duff K, | Barker R, | Nance M, | Beglinger L, | Moser D, | Williams JK, | Simpson S, | Langbehn D, | van Kammen DP, | Predict-Hd Investigators of the Huntington Study Group |

Challenges assessing clinical endpoints in early Huntington disease

Mov Disord

2595-603

25

(15):

20101115

PMID:20623772

DOI: 10.1002/mds.23337

PMCID: 2978744

Miller JP, | Holcomb J, | Al-Ramahi I, | de Haro M, | Gafni J, | Zhang N, | Kim E, | Sanhueza M, | Torcassi C, | Kwak S, | Botas J, | Hughes RE, | Ellerby LM |

Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease

Neuron

199-212

67

(2):

20100729

PMID:20670829

DOI: 10.1016/j.neuron.2010.06.021

PMCID: 3098887

Schaertl S, | Prime M, | Wityak J, | Dominguez C, | Munoz-Sanjuan I, | Pacifici RE, | Courtney S, | Scheel A, | Macdonald D |

A profiling platform for the characterization of transglutaminase 2 (TG2) inhibitors

J Biomol Screen

478-87

15

(5):

20100601

PMID:20395409

DOI: 10.1177/1087057110366035

PMCID:

Paulsen JS, | Nopoulos PC, | Aylward E, | Ross CA, | Johnson H, | Magnotta VA, | Juhl A, | Pierson RK, | Mills J, | Langbehn D, | Nance M, | Predict-Hd Investigators, | Coordinators of the Huntington`s Study Group |

Striatal and white matter predictors of estimated diagnosis for Huntington disease

Brain Res Bull

201-7

82

(3-4):

20100531

PMID:20385209

DOI: 10.1016/j.brainresbull.2010.04.003

PMCID: 2892238

Stoffers D, | Sheldon S, | Kuperman JM, | Goldstein J, | Corey-Bloom J, | Aron AR |

Contrasting gray and white matter changes in preclinical Huntington disease: an MRI study

Neurology

1208-16

74

(15):

20100413

PMID:20385893

DOI: 10.1212/WNL.0b013e3181d8c20a

PMCID: 2872799

Langbehn DR, | Hayden MR, | Paulsen JS, | Predict-Hd Investigators of the Huntington Study Group |

CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches

Am J Med Genet B Neuropsychiatr Genet

397-408

153B

(2):

20100305

PMID:19548255

DOI: 10.1002/ajmg.b.30992

PMCID: 3048807

Paulsen JS |

Early detection of Huntington disease

Future Neurol

5

(1):

20100101

PMID:24348095

DOI: 10.2217/fnl.09.78

PMCID: 3860286

Duff K, | Paulsen J, | Mills J, | Beglinger LJ, | Moser DJ, | Smith MM, | Langbehn D, | Stout J, | Queller S, | Harrington DL, | PREDICT-HD Investigators and Coordinators of the Huntington Study Group |

Mild cognitive impairment in prediagnosed Huntington disease

Neurology

500-7

75

(6):

20100101

PMID:20610833

DOI: 10.1212/WNL.0b013e3181eccfa2

PMCID: 2918475

Duff K, | Paulsen JS, | Beglinger LJ, | Langbehn DR, | Wang C, | Stout JC, | Ross CA, | Aylward E, | Carlozzi NE, | Queller S, | Predict-HD Investigators of the Huntington Study Group |

"Frontal" behaviors before the diagnosis of Huntington's disease and their relationship to markers of disease progression: evidence of early lack of awareness

J Neuropsychiatry Clin Neurosci

196-207

22

(2):

20100101

PMID:20463114

DOI: 10.1176/jnp.2010.22.2.196

PMCID: 2871328

Bechtel N, | Scahill RI, | Rosas HD, | Acharya T, | van den Bogaard SJ, | Jauffret C, | Say MJ, | Sturrock A, | Johnson H, | Onorato CE, | Salat DH, | Durr A, | Leavitt BR, | Roos RA, | Landwehrmeyer GB, | Langbehn DR, | Stout JC, | Tabrizi SJ, | Reilmann R |

Tapping linked to function and structure in premanifest and symptomatic Huntington disease

Neurology

2150-60

75

(24):

20100101

PMID:21068430

DOI: 10.1212/WNL.0b013e3182020123

PMCID: 3013584

Sturrock A, | Laule C, | Decolongon J, | Dar Santos R, | Coleman AJ, | Creighton S, | Bechtel N, | Reilmann R, | Hayden MR, | Tabrizi SJ, | Mackay AL, | Leavitt BR |

Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease

Neurology

1702-10

75

(19):

20100101

PMID:21060093

DOI: 10.1212/WNL.0b013e3181fc27e4

PMCID: 3033605

2009

Gu X, | Greiner ER, | Mishra R, | Kodali R, | Osmand A, | Finkbeiner S, | Steffan JS, | Thompson LM, | Wetzel R, | Yang XW |

Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice

Neuron

828-40

64

(6):

20091224

PMID:20064390

DOI: 10.1016/j.neuron.2009.11.020

PMCID: 2807408

Biglan KM, | Ross CA, | Langbehn DR, | Aylward EH, | Stout JC, | Queller S, | Carlozzi NE, | Duff K, | Beglinger LJ, | Paulsen JS, | Predict-Hd Investigators of the Huntington Study Group |

Motor abnormalities in premanifest persons with Huntington's disease: the PREDICT-HD study

Mov Disord

1763-72

24

(12):

20090915

PMID:19562761

DOI: 10.1002/mds.22601

PMCID: 3048804

Tabrizi SJ, | Langbehn DR, | Leavitt BR, | Roos RAC, | Durr A, | Craufurd D, | Kennard C, | Hicks SL, | Fox NC, | Scahill RI, | Borowsky B, | Tobin A J, | Rosas HD, | Johnson H, | Reilmann R, | Landwehrmeyer B, | Stout JC |

Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data

The Lancet Neurology

791-801

8

(9):

20090901

PMID:19646924

DOI: 10.1016/s1474-4422(09)70170-x

PMCID: 3725974

Menalled L, | El-Khodor BF, | Patry M, | Suarez-Farinas M, | Orenstein SJ, | Zahasky B, | Leahy C, | Wheeler V, | Yang XW, | MacDonald M, | Morton AJ, | Bates G, | Leeds J, | Park L, | Howland D, | Signer E, | Tobin A, | Brunner D |

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

Neurobiol Dis

319-36

35

(3):

20090901

PMID:19464370

DOI: 10.1016/j.nbd.2009.05.007

PMCID: 2728344

Liu Y, | Lacson R, | Cassaday J, | Ross DA, | Kreamer A, | Hudak E, | Peltier R, | McLaren D, | Munoz-Sanjuan I, | Santini F, | Strulovici B, | Ferrer M |

Identification of small-molecule modulators of mouse SVZ progenitor cell proliferation and differentiation through high-throughput screening

J Biomol Screen

319-29

14

(4):

20090401

PMID:19403915

DOI: 10.1177/1087057109332596

PMCID:

Weaver KE, | Richards TL, | Liang O, | Laurino MY, | Samii A, | Aylward EH |

Longitudinal diffusion tensor imaging in Huntington's Disease

Exp Neurol

525-9

216

(2):

20090401

PMID:19320010

DOI: 10.1016/j.expneurol.2008.12.026

PMCID:

Kloppel S, | Chu C, | Tan GC, | Draganski B, | Johnson H, | Paulsen JS, | Kienzle W, | Tabrizi SJ, | Ashburner J, | Frackowiak RS, | PREDICT-HD Investigators of the Huntington Study Group |

Automatic detection of preclinical neurodegeneration: presymptomatic Huntington disease

Neurology

426-31

72

(5):

20090101

PMID:19188573

DOI: 10.1212/01.wnl.0000341768.28646.b6

PMCID: 2817939

Munoz-Sanjuan, I |

Glial progenitor cell transplantation and the generation of chimeric animal models with human brain cells: implications for novel therapeutics

Expert Opin Ther Pat

1639-46

19

(12):

20090101

PMID:19939186

DOI:

PMCID:

2008

Southwell AL, | Khoshnan A, | Dunn DE, | Bugg CW, | Lo DC, | Patterson PH |

Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity

J Neurosci

9013-20

28

(36):

20080903

PMID:18768695

DOI: 10.1523/JNEUROSCI.2747-08.2008

PMCID: 2633448

Paulsen JS, | Langbehn DR, | Stout JC, | Aylward E, | Ross CA, | Nance M, | Guttman M, | Johnson S, | MacDonald M, | Beglinger LJ, | Duff K, | Kayson E, | Biglan K, | Shoulson I, | Oakes D, | Hayden M, | Predict-HD Investigators, | Coordinators of the Huntington Study Group |

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

J Neurol Neurosurg Psychiatry

874-80

79

(8):

20080801

PMID:18096682

DOI: 10.1136/jnnp.2007.128728

PMCID: 2569211

Cho SJ, | Sun Y |

Visual exploration of structure-activity relationship using maximum common framework

J Comput Aided Mol Des

571-8

22

(8):

20080801

PMID:18338222

DOI: 10.1007/s10822-008-9206-7

PMCID:

Ciamei A, | Morton AJ |

Rigidity in social and emotional memory in the R6/2 mouse model of Huntington's disease

Neurobiol Learn Mem

533-44

89

(4):

20080501

PMID:18069020

DOI: 10.1016/j.nlm.2007.10.009

PMCID:

Beglinger LJ, | Paulsen JS, | Watson DB, | Wang C, | Duff K, | Langbehn DR, | Moser DJ, | Paulson HL, | Aylward EH, | Carlozzi NE, | Queller S, | Stout JC |

Obsessive and compulsive symptoms in prediagnosed Huntington's disease

J Clin Psychiatry

1758-65

69

(11):

20080101

PMID:19012814

DOI:

PMCID:

2007

Duff K, | Paulsen JS, | Beglinger LJ, | Langbehn DR, | Stout JC, | Predict-HD Investigators of the Huntington Study Group |

Psychiatric symptoms in Huntington's disease before diagnosis: the predict-HD study

Biol Psychiatry

1341-6

62

(12):

20071215

PMID:17481592

DOI: 10.1016/j.biopsych.2006.11.034

PMCID:

DiFiglia M, | Sena-Esteves M, | Chase K, | Sapp E, | Pfister E, | Sass M, | Yoder J, | Reeves P, | Pandey RK, | Rajeev KG, | Manoharan M, | Sah DW, | Zamore PD, | Aronin N |

Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits

Proc Natl Acad Sci USA

17204-9

104

(43):

20071023

PMID:17940007

DOI: 10.1073/pnas.0708285104

PMCID: 2040405

Johnson SA, | Stout JC, | Solomon AC, | Langbehn DR, | Aylward EH, | Cruce CB, | Ross CA, | Nance M, | Kayson E, | Julian-Baros E, | Hayden MR, | Kieburtz K, | Guttman M, | Oakes D, | Shoulson I, | Beglinger L, | Duff K, | Penziner E, | Paulsen JS, | Predict-HD Investigators of the Huntington Study Group |

Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease

Brain

1732-44

130

(Pt 7):

20070701

PMID:17584778

DOI: 10.1093/brain/awm107

PMCID:

Solomon AC, | Stout JC, | Johnson SA, | Langbehn DR, | Aylward EH, | Brandt J, | Ross CA, | Beglinger L, | Hayden MR, | Kieburtz K, | Kayson E, | Julian-Baros E, | Duff K, | Guttman M, | Nance M, | Oakes D, | Shoulson I, | Penziner E, | Paulsen JS, | Predict-HD Investigators of the Huntington Study Group |

Verbal episodic memory declines prior to diagnosis in Huntington's disease

Neuropsychologia

1767-76

45

(8):

20070409

PMID:17303196

DOI: 10.1016/j.neuropsychologia.2006.12.015

PMCID: 2570315

Beglinger LJ, | Langbehn DR, | Duff K, | Stierman L, | Black DW, | Nehl C, | Anderson K, | Penziner E, | Paulsen JS, | Huntington Study Group Investigators |

Probability of obsessive and compulsive symptoms in Huntington's disease

Biol Psychiatry

415-8

61

(3):

20070201

PMID:16839521

DOI: 10.1016/j.biopsych.2006.04.034

PMCID:

2006

Van Raamsdonk JM, | Gibson WT, | Pearson J, | Murphy Z, | Lu G, | Leavitt BR, | Hayden MR |

Body weight is modulated by levels of full-length huntingtin

Hum Mol Genet

1513-23

15

(9):

20060501

PMID:16571604

DOI: 10.1093/hmg/ddl072

PMCID:

Paulsen JS, | Hayden M, | Stout JC, | Langbehn DR, | Aylward E, | Ross CA, | Guttman M, | Nance M, | Kieburtz K, | Oakes D, | Shoulson I, | Kayson E, | Johnson S, | Penziner E, | Predict-HD Investigators of the Huntington Study Group |

Preparing for preventive clinical trials: the Predict-HD study

Arch Neurol

883-90

63

(6):

20060101

PMID:16769871

DOI: 10.1001/archneur.63.6.883

PMCID:

2004

Paulsen JS, | Zimbelman JL, | Hinton SC, | Langbehn DR, | Leveroni CL, | Benjamin ML, | Reynolds NC, | Rao SM |

fMRI biomarker of early neuronal dysfunction in presymptomatic Huntington's disease

AJNR Am J Neuroradiol

1715-21

25

(10):

20041102

PMID:15569736

DOI:

PMCID: