Scientific Publications

Listed here are over 980 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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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
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
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
5
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
Biochemistry
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
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
Wójtowicz AM, Dvorzhak A, Semtner M, Grantyn R
Reduced tonic inhibition in striatal output neurons from Huntington mice due to loss of astrocytic GABA release through GAT-3
Front Neural Circuits
October 26, 2013
7 (188)
PMID: 24324407
PMCID: 3840359
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
Aylward EH, Harrington DL, Mills JA, Nopoulos PC, Ross CA, Long JD, Liu D, Westervelt HK, Paulsen JS, PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Regional atrophy associated with cognitive and motor function in prodromal Huntington disease
J Huntingtons Dis
October 1, 2013
2 (4) :477-89
PMID: 25062732
PMCID: 4412155
Liu J, Pendergraff H, Narayanannair KJ, Lackey JG, Kuchimanchi S, Rajeev KG, Manoharan M, Hu J, Corey DR
RNA duplexes with abasic substitutions are potent and allele-selective inhibitors of huntingtin and ataxin-3 expression
Nucleic Acids Res
October 1, 2013
41 (18) :8788-801
PMID: 23887934
PMCID: 3794577
Mason RP, Casu M, Butler N, Breda C, Campesan S, Clapp J, Green EW, Dhulkhed D, Kyriacou CP, Giorgini F
Glutathione peroxidase activity is neuroprotective in models of Huntington's disease
Nat Genet
October 1, 2013
45 (10) :1249-54
PMID: 23974869
PMCID: 4040417
Georgiou-Karistianis N, Poudel GR, Dominguez DJf, Langmaid R, Gray MA, Churchyard A, Chua P, Borowsky B, Egan GF, Stout JC
Functional and connectivity changes during working memory in Huntington`s disease: 18 month longitudinal data from the IMAGE-HD study
Brain Cogn
October 1, 2013
83 (1) :80-91
PMID: 23938592
Epping EA, Mills JA, Beglinger LJ, Fiedorowicz JG, Craufurd D, Smith MM, Groves M, Bijanki KR, Downing N, Williams JK, Long JD, Paulsen JS, Predict-Hd Investigators, Coordinators of the Huntington Study Group
Characterization of depression in prodromal Huntington disease in the neurobiological predictors of HD (PREDICT-HD) study
J Psychiatr Res
October 1, 2013
47 (10) :1423-31
PMID: 23790259
PMCID: PMC3808084
Moscovitch-Lopatin M, Goodman RE, Eberly S, Ritch JJ, Rosas HD, Matson S, Matson W, Oakes D, Young AB, Shoulson I, Hersch SM, Huntington Study Group PHAROS Investigators
HTRF analysis of soluble huntingtin in PHAROS PBMCs
Neurology
September 24, 2013
81 (13) :1134-40
PMID: 23966247
PMCID: 3795598
McCusker EA, Gunn DG, Epping EA, Loy CT, Radford K, Griffith J, Mills JA, Long JD, Paulsen JS, PREDICT-HD Investigators of the Huntington Study Group
Unawareness of motor phenoconversion in Huntington disease
Neurology
September 24, 2013
81 (13) :1141-7
PMID: 23966256
PMCID: 3795599
Domínguez D JF, Egan GF, Gray MA, Poudel GR, Churchyard A, Chua P, Stout JC, Georgiou-Karistianis N
Multi-modal neuroimaging in premanifest and early Huntington's disease: 18 month longitudinal data from the IMAGE-HD study
PLoS One
September 16, 2013
8 (9) :e74131
PMID: 24066104
PMCID: 3774648
Sbodio JI, Paul BD, Machamer CE, Snyder SH
Golgi protein ACBD3 mediates neurotoxicity associated with Huntington's disease
Cell Rep
September 12, 2013
4 (5) :890-897
PMID: 24012756
PMCID: 3801179
Lee CY, Cantle JP, Yang XW
Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis
FEBS J
September 2, 2013
280 (18) :4382-94
PMID: 23829302
PMCID: 3770892
Tang CC, Feigin A, Ma Y, Habeck C, Paulsen JS, Leenders KL, Teune LK, van Oostrom JC, Guttman M, Dhawan V, Eidelberg D
Metabolic network as a progression biomarker of premanifest Huntington's disease
J Clin Invest
September 2, 2013
123 (9) :4076-88
PMID: 23985564
PMCID: 3754266
Maillard MC, Dominguez C, Gemkow MJ, Krieger F, Park H, Schaertl S, Winkler D, Munoz-Sanjuan I
A label-free LC/MS/MS-based enzymatic activity assay for the detection of genuine caspase inhibitors and SAR development
J Biomol Screen
September 1, 2013
18 (8) :868-78
PMID: 23796689