Scientific Publications

Listed here are over 500 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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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
January 1, 2015
4 :e266
PMID: 26623938
Carroll JB, Bates GP, Steffan J, Saft C, Tabrizi SJ
Treating the whole body in Huntington`s disease
The Lancet Neurology
January 1, 2015
14 (11) :1135-1142
PMID: 26466780
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
January 1, 2015
4 (2) :141-8
PMID: 26397895
Wood NI, Morton AJ
Social behaviour is impaired in the R6/2 mouse model of Huntington's disease
J Huntingtons Dis
January 1, 2015
4 (1) :61-73
PMID: 26333258
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
January 1, 2015
4 (1) :37-47
PMID: 26333256
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
January 1, 2015
21 (1) :44064
PMID: 26304055
PMCID: PMC4549971
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
January 1, 2015
10 (8) :e0134465
PMID: 26295712
PMCID: PMC4546654
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
January 1, 2015
9 :32-43
PMID: 26288754
PMCID: PMC4536305
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
January 1, 2015
4 :e245
PMID: 26125484
PMCID: PMC4561652
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
January 1, 2015
74 (6) :527-37
PMID: 25978848
PMCID: PMC4435838
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
January 1, 2015
10 (4) :e0123527
PMID: 25859666
PMCID: 4393127
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
January 1, 2015
27 (1) :59-64
PMID: 25716488
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
January 1, 2015
10 (2) :e0117593
PMID: 25659157
PMCID: 4319930
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
January 1, 2015
27 (1) :44029
PMID: 25311499
PMCID: PMC4301197
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
January 1, 2015
36 (1) :110-9
PMID: 25139578
Mrzljak L, Munoz-Sanjuan I
Therapeutic strategies for Huntington`s Disease
Curr Top Behav Neurosci
January 1, 2015
22 :161-201
PMID: 24277342
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
January 1, 2015
220 (1) :501-12
PMID: 24240602
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
December 1, 2014
13 (12) :1193-201
PMID: 25453459
PMCID: 4373455
Ochaba J, Lukacsovich T, Csikos G, Zheng S, Margulis J, Salazar L, Mao K, Lau AL, Yeung SY, Humbert S, Saudou F, Klionsky DJ, Finkbeiner S, Zeitlin SO, Marsh JL, Housman DE, Thompson LM, Steffan JS
Potential function for the Huntingtin protein as a scaffold for selective autophagy
Proc Natl Acad Sci USA
November 24, 2014
111 (47) :16889-94
PMID: 25385587
PMCID: PMC4250109
Yao J, Ong SE, Bajjalieh S
Huntingtin is associated with cytomatrix proteins at the presynaptic terminal
Mol Cell Neurosci
November 1, 2014
63 :96-100
PMID: 25461618
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
October 15, 2014
8 :198
PMID: 25360089
PMCID: PMC4197654
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
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
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
July 2, 2014
83 (1) :178-88
PMID: 24991961
PMCID: PMC4131293
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
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
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
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
March 1, 2014
29 (3) :401-5
PMID: 24375941
PMCID: 3960314
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
February 1, 2014
19 (2) :191-204
PMID: 24196395
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
February 1, 2014
29 (2) :169-76
PMID: 23861335
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
January 1, 2014
3 (4) :355-63
PMID: 25575957
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
January 1, 2014
9 (12) :e112262
PMID: 25464275
PMCID: PMC4251833
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
January 1, 2014
3 (2) :159-74
PMID: 25062859