Scientific Publications

Listed here are over 1000 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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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
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
Brain
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