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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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
Neuron
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
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
January 1, 2014
3 (2) :139-44
PMID: 25062857
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
January 1, 2014
9 (7) :e102449
PMID: 25050979
PMCID: PMC4106781
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
January 1, 2014
9 (6) :e99520
PMID: 24955833
PMCID: PMC4067284
Cachope R, Cheer JF
Local control of striatal dopamine release
Front Behav Neurosci
January 1, 2014
8 :188
PMID: 24904339
PMCID: PMC4033078
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
January 1, 2014
9 (5) :e96854
PMID: 24816435
PMCID: PMC4016121
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
January 1, 2014
6 :78
PMID: 24795630
PMCID: PMC4000999
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
January 1, 2014
6
PMID: 24558637
PMCID: PMC3923778
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
January 1, 2014
9 (2) :e87923
PMID: 24503862
PMCID: PMC3913682
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
December 27, 2013
56 (24) :9934-54
PMID: 24261862
Franciosi S, Shim Y, Lau M, Hayden MR, Leavitt BR
A systematic review and meta-analysis of clinical variables used in Huntington disease research
Mov Disord
December 2, 2013
28 (14) :1987-94
PMID: 24142393
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
December 1, 2013
60 :89-107
PMID: 23969239
PMCID: PMC3808190
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
November 1, 2013
11 (11) :e1001717
PMID: 24302884
PMCID: PMC3841096
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
November 1, 2013
28 (13) :1793-800
PMID: 24038576
Ellison SM, Trabalza A, Tisato V, Pazarentzos E, Lee S, Papadaki V, Goniotaki D, Morgan S, Mirzaei N, Mazarakis ND
Dose-dependent neuroprotection of VEGF₁₆₅ in Huntington's disease striatum
Mol Ther
October 1, 2013
21 (10) :1862-75
PMID: 23799534
PMCID: 4059596