Scientific Publications

Listed here are over 1,045 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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Wild EJ, Tabrizi SJ
Targets for future clinical trials in Huntington's disease: what's in the pipeline?
Mov Disord
September 15, 2014
29 (11) :1434-45
PMID: 25155142
PMCID: 4265300
Sampaio C, Borowsky B, Reilmann R
Clinical trials in Huntington`s disease: Interventions in early clinical development and newer methodological approaches
Mov Disord
September 15, 2014
29 (11) :1419-28
PMID: 25216371
Howland DS, Munoz-Sanjuan I
Mind the gap: models in multiple species needed for therapeutic development in Huntington`s disease
Mov Disord
September 15, 2014
29 (11) :1397-403
PMID: 25155258
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
September 1, 2014
29 (10) :1281-8
PMID: 25209258
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
September 1, 2014
32 (9) :871-3
PMID: 25203032
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
September 1, 2014
23 (17) :4510-27
PMID: 24728190
Kozak R, Campbell BM, Strick CA, Horner W, Hoffmann WE, Kiss T, Chapin DS, McGinnis D, Abbott AL, Roberts BM, Fonseca K, Guanowsky V, Young DA, Seymour PA, Dounay A, Hajos M, Williams GV, Castner SA
Reduction of brain kynurenic acid improves cognitive function
J Neurosci
August 6, 2014
34 (32) :10592-602
PMID: 25100593
PMCID: 6802596
Georgiou-Karistianis N, Long JD, Lourens SG, Stout JC, Mills JA, Paulsen JS, Predict-Hd Investigators and Coordinators Of The Huntington Study Group
Movement sequencing in Huntington disease
World J Biol Psychiatry
August 1, 2014
15 (6) :459-71
PMID: 24678867
PMCID: 4389285
Butland SL, Sanders SS, Schmidt ME, Riechers SP, Lin DT, Martin DD, Vaid K, Graham RK, Singaraja RR, Wanker EE, Conibear E, Hayden MR
The palmitoyl acyltransferase HIP14 shares a high proportion of interactors with huntingtin: implications for a role in the pathogenesis of Huntington's disease
Hum Mol Genet
August 1, 2014
23 (15) :4142-60
PMID: 24705354
PMCID: 4082372
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
August 1, 2014
10 (8) :e1004550
PMID: 25101683
PMCID: PMC4125112
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
August 1, 2014
22 (8) :958-63
PMID: 24327189
PMCID: PMC4350592
Dominguez C, Munoz-Sanjuan I
Foundation-directed therapeutic development in Huntington`s disease
J Med Chem
July 10, 2014
57 (13) :5479-88
PMID: 24432836
McKinstry SU, Karadeniz YB, Worthington AK, Hayrapetyan VY, Ozlu MI, Serafin-Molina K, Risher WC, Ustunkaya T, Dragatsis I, Zeitlin S, Yin HH, Eroglu C
Huntingtin is required for normal excitatory synapse development in cortical and striatal circuits
J Neurosci
July 9, 2014
34 (28) :9455-72
PMID: 25009276
PMCID: 4087216
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
July 2, 2014
83 (1) :178-88
PMID: 24991961
PMCID: PMC4131293
Morton AJ, Rudiger SR, Wood NI, Sawiak SJ, Brown GC, Mclaughlan CJ, Kuchel TR, Snell RG, Faull RL, Bawden CS
Early and progressive circadian abnormalities in Huntington's disease sheep are unmasked by social environment
Hum Mol Genet
July 1, 2014
23 (13) :3375-83
PMID: 24488771
Andre R, Scahill RI, Haider S, Tabrizi SJ
Biomarker development for Huntington's disease
Drug Discov Today
July 1, 2014
19 (7) :972-9
PMID: 24632006
Sepers MD, Raymond LA
Mechanisms of synaptic dysfunction and excitotoxicity in Huntington`s disease
Drug Discov Today
July 1, 2014
19 (7) :990-6
PMID: 24603212
Signer, E
Through postdoc eyes
Res Microbiol
June 1, 2014
165 (5) :346-7
PMID: 24859142
Georgiou-Karistianis N, Stout JC, Domínguez D JF, Carron SP, Ando A, Churchyard A, Chua P, Bohanna I, Dymowski AR, Poudel G, Egan GF
Functional magnetic resonance imaging of working memory in Huntington's disease: cross-sectional data from the IMAGE-HD study
Hum Brain Mapp
May 1, 2014
35 (5) :1847-64
PMID: 23913754
PMCID: 6869353
Paul BD, Sbodio JI, Xu R, Vandiver MS, Cha JY, Snowman AM, Snyder SH
Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington's disease
Nature
May 1, 2014
509 (7498) :96-100
PMID: 24670645
PMCID: 4349202
Poudel GR, Stout JC, Domínguez D JF, Salmon L, Churchyard A, Chua P, Georgiou-Karistianis N, Egan GF
White matter connectivity reflects clinical and cognitive status in Huntington's disease
Neurobiol Dis
May 1, 2014
65 :180-7
PMID: 24480090
Tong X, Ao Y, Faas GC, Nwaobi SE, Xu J, Haustein MD, Anderson MA, Mody I, Olsen ML, Sofroniew MV, Khakh BS
Astrocyte Kir4.1 ion channel deficits contribute to neuronal dysfunction in Huntington's disease model mice
Nat Neurosci
May 1, 2014
17 (5) :694-703
PMID: 24686787
PMCID: 4064471
Wang N, Gray M, Lu XH, Cantle JP, Holley SM, Greiner E, Gu X, Shirasaki D, Cepeda C, Li Y, Dong H, Levine MS, Yang XW
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease
Nat Med
May 1, 2014
20 (5) :536-41
PMID: 24784230
PMCID: 4067603
Yu A, Shibata Y, Shah B, Calamini B, Lo DC, Morimoto RI
Protein aggregation can inhibit clathrin-mediated endocytosis by chaperone competition
Proc Natl Acad Sci USA
April 15, 2014
111 (15) :E1481-90
PMID: 24706768
PMCID: 3992642
An MC, O’Brien RN, Zhang N, Patra BN, De La Cruz M, Ray A, Ellerby LM
Polyglutamine disease modeling: epitope based screen for homologous recombination using CRISPR/Cas9 system
PLoS Curr
April 15, 2014
6 :ecurrents.hd
PMID: 24761311
PMCID: 3994193
Kolodziejczyk K, Parsons MP, Southwell AL, Hayden MR, Raymond LA
Striatal synaptic dysfunction and hippocampal plasticity deficits in the Hu97/18 mouse model of Huntington disease
PLoS One
April 11, 2014
9 (4) :e94562
PMID: 24728353
PMCID: 3984157
Matsui JT, Vaidya JG, Johnson HJ, Magnotta VA, Long JD, Mills JA, Lowe MJ, Sakaie KE, Rao SM, Smith MM, Paulsen JS
Diffusion weighted imaging of prefrontal cortex in prodromal Huntington's disease
Hum Brain Mapp
April 2, 2014
35 (4) :1562-73
PMID: 23568433
PMCID: 3775984
Zeef DH, Jahanshahi A, Vlamings R, Casaca-Carreira J, Santegoeds RG, Janssen ML, Oosterloo M, Temel Y
An experimental model for Huntington's chorea?
Behav Brain Res
April 1, 2014
262 :31-4
PMID: 24412685
Crotti A, Benner C, Kerman BE, Gosselin D, Lagier-Tourenne C, Zuccato C, Cattaneo E, Gage FH, Cleveland DW, Glass CK
Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors
Nat Neurosci
April 1, 2014
17 (4) :513-21
PMID: 24584051
PMCID: 4113004
Skodda S, Schlegel U, Hoffmann R, Saft C
Impaired motor speech performance in Huntington's disease
J Neural Transm (Vienna)
April 1, 2014
121 (4) :399-407
PMID: 24221215
Jones R, Stout JC, Labuschagne I, Say M, Justo D, Coleman A, Dumas EM, Hart E, Owen G, Durr A, Leavitt BR, Roos R, O’Regan A, Langbehn D, Tabrizi SJ, Frost C
The potential of composite cognitive scores for tracking progression in Huntington's disease
J Huntingtons Dis
April 1, 2014
3 (2) :197-207
PMID: 25062862
Jiang H, Sun YM, Hao Y, Yan YP, Chen K, Xin SH, Tang YP, Li XH, Jun T, Chen YY, Liu ZJ, Wang CR, Li H, Pei Z, Shang HF, Zhang BR, Gu WH, Wu ZY, Tang BS, Burgunder JM, Chinese HD Network
Huntingtin gene CAG repeat numbers in Chinese patients with Huntington's disease and controls
Eur J Neurol
April 1, 2014
21 (4) :637-42
PMID: 24471773
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
April 1, 2014
10 (4) :204-16
PMID: 24614516
Cuesta M, Aungier J, Morton AJ
Behavioral therapy reverses circadian deficits in a transgenic mouse model of Huntington's disease
Neurobiol Dis
March 26, 2014
63 :85-91
PMID: 24269914
Cole JH, Farmer RE, Rees EM, Johnson HJ, Frost C, Scahill RI, Hobbs NZ
Test-retest reliability of diffusion tensor imaging in Huntington's disease
PLoS Curr
March 21, 2014
6
PMID: 24672743
PMCID: 3962450
Tourette C, Li B, Bell R, O’Hare S, Kaltenbach LS, Mooney SD, Hughes RE
A large scale Huntingtin protein interaction network implicates Rho GTPase signaling pathways in Huntington disease
J Biol Chem
March 7, 2014
289 (10) :6709-26
PMID: 24407293
PMCID: 3945331
Long JD, Paulsen JS, Marder K, Zhang Y, Kim JI, Mills JA, Researchers of the PREDICT-HD Huntington’s Study Group
Tracking motor impairments in the progression of Huntington's disease
Mov Disord
March 1, 2014
29 (3) :311-9
PMID: 24150908
PMCID: 4219558
Hart EP, Dumas EM, Schoonderbeek A, Wolthuis SC, van Zwet EW, Roos RA
Motor dysfunction influence on executive functioning in manifest and premanifest Huntington's disease
Mov Disord
March 1, 2014
29 (3) :320-6
PMID: 24500841
Novak MJ, Seunarine KK, Gibbard CR, Hobbs NZ, Scahill RI, Clark CA, Tabrizi SJ
White matter integrity in premanifest and early Huntington's disease is related to caudate loss and disease progression
Cortex
March 1, 2014
52 :98-112
PMID: 24405816
Padowski JM, Weaver KE, Richards TL, Laurino MY, Samii A, Aylward EH, Conley KE
Neurochemical correlates of caudate atrophy in Huntington's disease
Mov Disord
March 1, 2014
29 (3) :327-35
PMID: 24442623
PMCID: 3960319