2018

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

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20181203

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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

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20180928

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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

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20180912

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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

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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

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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

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20180801

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PMCID:

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

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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

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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

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PMID:29912367

DOI: 10.1093/hmg/ddy141

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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

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20180629

PMID:30013509

DOI: 10.3389/fneur.2018.00516

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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

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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

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20180606

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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

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20180601

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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

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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

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20180528

PMID:29804232

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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

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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

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20180524

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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

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20180522

PMID:29789657

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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

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20180519

PMID:29843247

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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

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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

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20180511

PMID:29750799

DOI: 10.1371/journal.pgen.1007274

PMCID:

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

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20180509

PMID:29984153

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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

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20180422

PMID:29682858

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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

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20180417

PMID:29660633

DOI: 10.1016/j.gaitpost.2018.03.039

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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

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20180402

PMID:29761120

DOI: 10.1002/acn3.558

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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

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20180330

PMID:29222609

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PMCID: 5845825

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

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PMID:29581148

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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

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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

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20180319

PMID:29554128

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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

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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

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20180313

PMID:29527479

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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

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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

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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

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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

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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

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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

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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

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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

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20180222

PMID:29125980

DOI: 10.1016/j.conb.2017.10.019

PMCID:

Rebec GV |

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

CNS Neurosci Ther

281-291

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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

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20180220

PMID:29205501

DOI: 10.1002/mds.27247

PMCID:

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

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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

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20180208

PMID:29432571

DOI: 10.1093/nar/gky037

PMCID:

Arnerić SP, | Kern VD, | Stephenson DT |

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

Biochem Pharmacol

291-306

151

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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

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20171212

PMID:29125493

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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

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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

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20171206

PMID:29212711

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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

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20171124

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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

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