Scientific Publications

Listed here are over 950 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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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
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
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
Träger U, Andre R, Lahiri N, Magnusson-Lind A, Weiss A, Grueninger S, McKinnon C, Sirinathsinghji E, Kahlon S, Pfister EL, Moser R, Hummerich H, Antoniou M, Bates GP, Luthi-Carter R, Lowdell MW, Björkqvist M, Ostroff GR, Aronin N, Tabrizi SJ
HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation
March 1, 2014
137 (Pt 3) :819-33
PMID: 24459107
PMCID: 3983408
Poudel GR, Egan GF, Churchyard A, Chua P, Stout JC, Georgiou-Karistianis N
Abnormal synchrony of resting state networks in premanifest and symptomatic Huntington disease: the IMAGE-HD study
J Psychiatry Neurosci
March 1, 2014
39 (2) :87-96
PMID: 24083458
PMCID: 3937285
Younes L, Ratnanather JT, Brown T, Aylward E, Nopoulos P, Johnson H, Magnotta VA, Paulsen JS, Margolis RL, Albin RL, Miller MI, Ross CA, PREDICT-HD Investigators and Coordinators of the HSG
Regionally selective atrophy of subcortical structures in prodromal HD as revealed by statistical shape analysis
Hum Brain Mapp
March 1, 2014
35 (3) :792-809
PMID: 23281100
PMCID: 3715588
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
Sanders SS, Mui KK, Sutton LM, Hayden MR
Identification of binding sites in huntingtin for the huntingtin interacting proteins HIP14 and HIP14L
PLoS One
February 28, 2014
9 (2) :e90669
PMID: 24651384
PMCID: 3947954
Mealer RG, Murray AJ, Shahani N, Subramaniam S, Snyder SH
Rhes, a striatal-selective protein implicated in Huntington disease, binds beclin-1 and activates autophagy
J Biol Chem
February 7, 2014
289 (6) :3547-54
PMID: 24324270
PMCID: 3916556
Sajjad MU, Green EW, Miller-Fleming L, Hands S, Herrera F, Campesan S, Khoshnan A, Outeiro TF, Giorgini F, Wyttenbach A
DJ-1 modulates aggregation and pathogenesis in models of Huntington's disease
Hum Mol Genet
February 1, 2014
23 (3) :755-66
PMID: 24070869
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
Buonincontri G, Wood NI, Puttick SG, Ward AO, Carpenter TA, Sawiak SJ, Morton AJ
Right ventricular dysfunction in the R6/2 transgenic mouse model of Huntington's disease is unmasked by dobutamine
J Huntingtons Dis
January 31, 2014
3 (1) :25-32
PMID: 24744818
PMCID: 3986496
Ness V, Bestgen AK, Saft C, Beste C
Changes in cognitive control in pre-manifest Huntington's disease examined using pre-saccadic EEG potentials - a longitudinal study
J Huntingtons Dis
January 1, 2014
3 (1) :33-43
PMID: 25062763
Muralidharan P, Fishbaugh J, Johnson HJ, Durrleman S, Paulsen JS, Gerig G, Fletcher PT
Diffeomorphic shape trajectories for improved longitudinal segmentation and statistics
Med Image Comput Comput Assist
January 1, 2014
17 :49-56
PMID: 25320781
PMCID: 4486086
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
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
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
Träger U, Magnusson A, Lahiri Swales N, Wild E, North J, Lowdell M, Björkqvist M
JAK/STAT signalling in Huntington's disease immune cells
PLoS Curr
December 13, 2013
PMID: 24459609
PMCID: 3871417
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
Dorsey ER, Brocht AF, Nichols PE, Darwin KC, Anderson KE, Beck CA, Singh S, Biglan KM, Shoulson I
Depressed mood and suicidality in individuals exposed to tetrabenazine in a large Huntington disease observational study
J Huntingtons Dis
December 1, 2013
2 (4) :509-15
PMID: 25062735
Crawford HE, Hobbs NZ, Keogh R, Langbehn DR, Frost C, Johnson H, Landwehrmeyer B, Reilmann R, Craufurd D, Stout JC, Durr A, Leavitt BR, Roos RA, Tabrizi SJ, Scahill RI, TRACK-HD Investigators
Corpus callosal atrophy in premanifest and early Huntington's disease
J Huntingtons Dis
December 1, 2013
2 (4) :517-26
PMID: 25062736
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
Busan S, Weeks KM
Role of context in RNA structure: flanking sequences reconfigure CAG motif folding in huntingtin exon 1 transcripts
November 19, 2013
52 (46) :8219-25
PMID: 24199621
PMCID: 4890576
Sawiak SJ, Wood NI, Williams GB, Morton AJ, Carpenter TA
Voxel-based morphometry with templates and validation in a mouse model of Huntington's disease
Magn Reson Imaging
November 1, 2013
31 (9) :1522-31
PMID: 23835187
PMCID: 3919157
Paulsen JS, Smith MM, Long JD, PREDICT HD investigators and Coordinators of the Huntington Study Group
Cognitive decline in prodromal Huntington Disease: implications for clinical trials
J Neurol Neurosurg Psychiatry
November 1, 2013
84 (11) :1233-9
PMID: 23911948
PMCID: 3795884
Gipson TA, Neueder A, Wexler NS, Bates GP, Housman D
Aberrantly spliced HTT, a new player in Huntington's disease pathogenesis
RNA Biol
November 1, 2013
10 (11) :1647-52
PMID: 24256709
PMCID: 3907474