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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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
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
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
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
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
Winkler D, Beconi M, Toledo-Sherman LM, Prime M, Ebneth A, Dominguez C, Munoz-Sanjuan I
Development of LC/MS/MS, high-throughput enzymatic and cellular assays for the characterization of compounds that inhibit kynurenine monooxygenase (KMO)
J Biomol Screen
September 1, 2013
18 (8) :879-89
PMID: 23690293
Delmaire C, Dumas EM, Sharman MA, van den Bogaard SJ, Valabregue R, Jauffret C, Justo D, Reilmann R, Stout JC, Craufurd D, Tabrizi SJ, Roos RA, Durr A, Lehericy S
The structural correlates of functional deficits in early huntington`s disease
Hum Brain Mapp
September 1, 2013
34 (9) :2141-53
PMID: 22438242
Lee JM, Galkina EI, Levantovsky RM, Fossale E, Anne Anderson M, Gillis T, Srinidhi Mysore J, Coser KR, Shioda T, Zhang B, Furia MD, Derry J, Kohane IS, Seong IS, Wheeler VC, Gusella JF, MacDonald ME
Dominant effects of the Huntington`s disease HTT CAG repeat length are captured in gene-expression data sets by a continuous analysis mathematical modeling strategy
Hum Mol Genet
August 15, 2013
22 (16) :3227-38
PMID: 23595883
PMCID: PMC3723309
Marco S, Giralt A, Petrovic MM, Pouladi MA, Martínez-Turrillas R, Martínez-Hernández J, Kaltenbach LS, Torres-Peraza J, Graham RK, Watanabe M, Luján R, Nakanishi N, Lipton SA, Lo DC, Hayden MR, Alberch J, Wesseling JF, Pérez-Otaño I
Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models
Nat Med
August 4, 2013
19 (8) :1030-38
PMID: 23852340
PMCID: 3936794
Divino V, Dekoven M, Warner JH, Giuliano J, Anderson KE, Langbehn D, Lee WC
The direct medical costs of Huntington`s disease by stage. A retrospective commercial and Medicaid claims data analysis
J Med Econ
August 1, 2013
16 (8) :1043-50
PMID: 23789925
Bonner-Jackson A, Long JD, Westervelt H, Tremont G, Aylward E, Paulsen JS, Predict-Hd Investigators, Coordinators of the Huntington Study Group
Cognitive reserve and brain reserve in prodromal Huntington`s disease
J Int Neuropsychol Soc
August 1, 2013
19 (7) :739-50
PMID: 23702309
PMCID: PMC3720793
Delli Carri A, Onorati M, Castiglioni V, Faedo A, Camnasio S, Toselli M, Biella G, Cattaneo E
Human pluripotent stem cell differentiation into authentic striatal projection neurons
Stem Cell Rev
August 1, 2013
9 (4) :461-74
PMID: 23625190
O’Rourke JG, Gareau JR, Ochaba J, Song W, Raskó T, Reverter D, Lee J, Monteys AM, Pallos J, Mee L, Vashishtha M, Apostol BL, Nicholson TP, Illes K, Zhu YZ, Dasso M, Bates GP, Difiglia M, Davidson B, Wanker EE, Marsh JL, Lima CD, Steffan JS, Thompson LM
SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation
Cell Rep
July 25, 2013
4 (2) :362-75
PMID: 23871671
PMCID: 3931302
Chen JY, Wang EA, Cepeda C, Levine MS
Dopamine imbalance in Huntington's disease: a mechanism for the lack of behavioral flexibility
Front Neurosci
July 4, 2013
7 :114
PMID: 23847463
PMCID: 3701870
Kantor S, Szabo L, Varga J, Cuesta M, Morton AJ
Progressive sleep and electroencephalogram changes in mice carrying the Huntington's disease mutation
July 2, 2013
136 (7) :2147-58
PMID: 23801737
Fisher SP, Black SW, Schwartz MD, Wilk AJ, Chen TM, Lincoln WU, Liu HW, Kilduff TS, Morairty SR
Longitudinal analysis of the electroencephalogram and sleep phenotype in the R6/2 mouse model of Huntington's disease
July 1, 2013
136 (7) :2159-72
PMID: 23801738
Bari M, Battista N, Valenza M, Mastrangelo N, Malaponti M, Catanzaro G, Centonze D, Finazzi-Agrò A, Cattaneo E, Maccarrone M
In vitro and in vivo models of Huntington's disease show alterations in the endocannabinoid system
July 1, 2013
280 (14) :3376-88
PMID: 23659592
Vellas B, Carrillo MC, Sampaio C, Brashear HR, Siemers E, Hampel H, Schneider LS, Weiner M, Doody R, Khachaturian Z, Cedarbaum J, Grundman M, Broich K, Giacobini E, Dubois B, Sperling R, Wilcock GK, Fox N, Scheltens P, Touchon J, Hendrix S, Andrieu S, Aisen P, EU US CTAD Task Force Members
Designing drug trials for Alzheimer`s disease: what we have learned from the release of the phase III antibody trials: a report from the EU/US/CTAD Task Force
Alzheimers Dement
July 1, 2013
9 (4) :438-44
PMID: 23809364
Tabrizi SJ, Scahill RI, Owen G, Durr A, Leavitt BR, Roos RA, Borowsky B, Landwehrmeyer B, Frost C, Johnson H, Craufurd D, Reilmann R, Stout JC, Langbehn DR, Track-Hd Investigators
Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington`s disease in the TRACK-HD study: analysis of 36-month observational data
Lancet Neurol
July 1, 2013
12 (7) :637-49
PMID: 23664844
Jiang M, Peng Q, Liu X, Jin J, Hou Z, Zhang J, Mori S, Ross CA, Ye K, Duan W
Small-molecule TrkB receptor agonists improve motor function and extend survival in a mouse model of Huntington's disease
Hum Mol Genet
June 15, 2013
22 (12) :2462-70
PMID: 23446639
PMCID: 3658168