Scientific Publications

Listed here are over 900 publications that describe some of the research funded (in whole or in part) by CHDI Foundation. You can also view just the subset of publications that have CHDI staff as authors.

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Orth M, Handley OJ, Schwenke C, Dunnett SB, Craufurd D, Ho AK, Wild E, Tabrizi SJ, Landwehrmeyer GB, Investigators of the European Huntington’s Disease Network
Observing Huntington's disease: the European Huntington's Disease Network's REGISTRY
PLoS Curr
September 28, 2010
2 :RRN1184
PMID: 20890398
PMCID: 2947793
Marrakchi-Kacem L, Delmaire C, Tucholka A, Roca P, Guevara P, Poupon F, Yelnik J, Durr A, Mangin JF, Lehericy S, Poupon C
Analysis of the striato-thalamo-cortical connectivity on the cortical surface to infer biomarkers of Huntington's disease
Med Image Comput Comput Assist
September 20, 2010
13 :217-24
PMID: 20879318
Perry GM, Tallaksen-Greene S, Kumar A, Heng MY, Kneynsberg A, van Groen T, Detloff PJ, Albin RL, Lesort M
Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease
Hum Mol Genet
September 1, 2010
19 (17) :3354-71
PMID: 20558522
PMCID: 2916705
Jahanshahi A, Vlamings R, Kaya AH, Lim LW, Janssen ML, Tan S, Visser-Vandewalle V, Steinbusch HW, Temel Y
Hyperdopaminergic status in experimental Huntington disease
J Neuropathol Exp Neurol
September 1, 2010
69 (9) :910-7
PMID: 20720506
Valenza M, Leoni V, Karasinska JM, Petricca L, Fan J, Carroll J, Pouladi MA, Fossale E, Nguyen HP, Riess O, MacDonald M, Wellington C, DiDonato S, Hayden M, Cattaneo E
Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes
J Neurosci
August 11, 2010
30 (32) :10844-50
PMID: 20702713
PMCID: 3842469
Kaltenbach LS, Bolton MM, Shah B, Kanju PM, Lewis GM, Turmel GJ, Whaley JC, Trask OJ Jr, Lo DC
Composite primary neuronal high-content screening assay for Huntington's disease incorporating non-cell-autonomous interactions
J Biomol Screen
August 1, 2010
15 (7) :806-19
PMID: 20581077
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
July 29, 2010
67 (2) :199-212
PMID: 20670829
PMCID: PMC3098887
Maywood ES, Fraenkel E, McAllister CJ, Wood N, Reddy AB, Hastings MH, Morton AJ
Disruption of peripheral circadian timekeeping in a mouse model of Huntington's disease and its restoration by temporally scheduled feeding
J Neurosci
July 28, 2010
30 (30) :10199-204
PMID: 20668203
PMCID: 6633357
Hobbs NZ, Henley SM, Ridgway GR, Wild EJ, Barker RA, Scahill RI, Barnes J, Fox NC, Tabrizi SJ
The progression of regional atrophy in premanifest and early Huntington's disease: a longitudinal voxel-based morphometry study
J Neurol Neurosurg Psychiatry
July 1, 2010
81 (7) :756-63
PMID: 19955112
Zuccato C, Valenza M, Cattaneo E
Molecular mechanisms and potential therapeutical targets in Huntington's disease
Physiol Rev
July 1, 2010
90 (3) :905-81
PMID: 20664076
Rowe KC, Paulsen JS, Langbehn DR, Duff K, Beglinger LJ, Wang C, O’Rourke JJ, Stout JC, Moser DJ
Self-paced timing detects and tracks change in prodromal Huntington disease
Neuropsychology
July 1, 2010
24 (4) :435-42
PMID: 20604618
PMCID: 2900808
Hobbs NZ, Barnes J, Frost C, Henley SM, Wild EJ, Macdonald K, Barker RA, Scahill RI, Fox NC, Tabrizi SJ
Onset and progression of pathologic atrophy in Huntington disease: a longitudinal MR imaging study
AJNR Am J Neuroradiol
June 1, 2010
31 (6) :1036-41
PMID: 20150305
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
June 1, 2010
15 (5) :478-87
PMID: 20395409
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
May 31, 2010
82 (43894) :201-7
PMID: 20385209
PMCID: PMC2892238
Crittenden JR, Dunn DE, Merali FI, Woodman B, Yim M, Borkowska AE, Frosch MP, Bates GP, Housman DE, Lo DC, Graybiel AM
CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease
Hum Mol Genet
May 1, 2010
19 (9) :1756-65
PMID: 20147317
PMCID: 2850620
Becanovic K, Pouladi MA, Lim RS, Kuhn A, Pavlidis P, Luthi-Carter R, Hayden MR, Leavitt BR
Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis
Hum Mol Genet
April 15, 2010
19 (8) :1438-52
PMID: 20089533
PMCID: 2846159
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
April 13, 2010
74 (15) :1208-16
PMID: 20385893
PMCID: 2872799
Menalled LB, Patry M, Ragland N, Lowden PA, Goodman J, Minnich J, Zahasky B, Park L, Leeds J, Howland D, Signer E, Tobin AJ, Brunner D
Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline
PLoS One
March 22, 2010
5 (3) :e9793
PMID: 20339553
PMCID: 2842438
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
March 5, 2010
153B (2) :397-408
PMID: 19548255
PMCID: PMC3048807
Wood NI, Carta V, Milde S, Skillings EA, McAllister CJ, Ang YL, Duguid A, Wijesuriya N, Afzal SM, Fernandes JX, Leong TW, Morton AJ
Responses to environmental enrichment differ with sex and genotype in a transgenic mouse model of Huntington's disease
PLoS One
February 12, 2010
5 (2) :e9077
PMID: 20174443
PMCID: 2820540
Yerkes S, Vesenka J, Kmiec EB
A stable G-quartet binds to a huntingtin protein fragment containing expanded polyglutamine tracks
J Neurosci Res
February 1, 2010
88 (2) :335-45
PMID: 19705452
Paulsen JS
Early detection of Huntington disease
Future Neurol
January 1, 2010
5 (1)
PMID: 24348095
PMCID: PMC3860286
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
January 1, 2010
75 (24) :2150-60
PMID: 21068430
PMCID: PMC3013584
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
January 1, 2010
75 (19) :1702-10
PMID: 21060093
PMCID: PMC3033605
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
January 1, 2010
75 (6) :500-7
PMID: 20610833
PMCID: PMC2918475
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
January 1, 2010
22 (2) :196-207
PMID: 20463114
PMCID: PMC2871328
Bradford J, Shin JY, Roberts M, Wang CE, Li XJ, Li S
Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms
Proc Natl Acad Sci USA
December 29, 2009
106 (52) :22480-5
PMID: 20018729
PMCID: 2799722
Thompson LM, Aiken CT, Kaltenbach LS, Agrawal N, Illes K, Khoshnan A, Martinez-Vincente M, Arrasate M, O’Rourke JG, Khashwji H, Lukacsovich T, Zhu YZ, Lau AL, Massey A, Hayden MR, Zeitlin SO, Finkbeiner S, Green KN, LaFerla FM, Bates G, Huang L, Patterson PH, Lo DC, Cuervo AM, Marsh JL, Steffan JS
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome
J Cell Biol
December 28, 2009
187 (7) :1083-99
PMID: 20026656
PMCID: 2806289
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
December 24, 2009
64 (6) :828-40
PMID: 20064390
PMCID: PMC2807408
Moffitt H, McPhail GD, Woodman B, Hobbs C, Bates GP
Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease
PLoS One
November 30, 2009
4 (11) :e8025
PMID: 19956633
PMCID: 2778556
Li X, Standley C, Sapp E, Valencia A, Qin ZH, Kegel KB, Yoder J, Comer-Tierney LA, Esteves M, Chase K, Alexander J, Masso N, Sobin L, Bellve K, Tuft R, Lifshitz L, Fogarty K, Aronin N, DiFiglia M
Mutant huntingtin impairs vesicle formation from recycling endosomes by interfering with Rab11 activity
Mol Cell Biol
November 1, 2009
29 (22) :6106-16
PMID: 19752198
PMCID: 2772576
Ciamei A, Morton AJ
Progressive imbalance in the interaction between spatial and procedural memory systems in the R6/2 mouse model of Huntington's disease
Neurobiol Learn Mem
October 12, 2009
92 (3) :417-28
PMID: 19524696
Hobbs NZ, Henley SM, Wild EJ, Leung KK, Frost C, Barker RA, Scahill RI, Barnes J, Tabrizi SJ, Fox NC
Automated quantification of caudate atrophy by local registration of serial MRI: evaluation and application in Huntington's disease
Neuroimage
October 1, 2009
47 (4) :1659-65
PMID: 19523522
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
September 15, 2009
24 (12) :1763-72
PMID: 19562761
PMCID: PMC3048804
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
September 1, 2009
8 (9) :791-801
PMID: 19646924
PMCID: PMC3725974
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
September 1, 2009
35 (3) :319-36
PMID: 19464370
PMCID: PMC2728344
Silvestroni A, Faull RL, Strand AD, Möller T
Distinct neuroinflammatory profile in post-mortem human Huntington's disease
Neuroreport
August 5, 2009
20 (12) :1098-103
PMID: 19590393
Pallier PN, Morton AJ
Management of sleep/wake cycles improves cognitive function in a transgenic mouse model of Huntington's disease
Brain Res
July 7, 2009
1279 :90-8
PMID: 19450569
Zuccato C, Cattaneo E
Brain-derived neurotrophic factor in neurodegenerative diseases
Nat Rev Neurol
June 1, 2009
5 (6) :311-22
PMID: 19498435
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
April 1, 2009
14 (4) :319-29
PMID: 19403915