Scientific Publications

Listed here are over 900 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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Zeef DH, van Goethem NP, Vlamings R, Schaper F, Jahanshahi A, Hescham S, von Hörsten S, Prickaerts J, Temel Y
Memory deficits in the transgenic rat model of Huntington's disease
Behav Brain Res
February 1, 2012
227 (1) :194-8
PMID: 22101303
Zeef DH, Vlamings R, Lim LW, Tan S, Janssen ML, Jahanshahi A, Hoogland G, Prickaerts J, Steinbusch HW, Temel Y
Motor and non-motor behaviour in experimental Huntington's disease
Behav Brain Res
January 15, 2012
226 (2) :435-9
PMID: 22001615
Neveklovska M, Clabough EB, Steffan JS, Zeitlin SO
Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice
J Huntingtons Dis
January 2, 2012
1 (1) :71-87
PMID: 22956985
PMCID: PMC3432989
Cuesta M, Aungier J, Morton AJ
The methamphetamine-sensitive circadian oscillator is dysfunctional in a transgenic mouse model of Huntington's disease
Neurobiol Dis
January 1, 2012
45 (1) :145-55
PMID: 21820053
Sarantos MR, Papanikolaou T, Ellerby LM, Hughes RE
Pizotifen activates ERK and provides neuroprotection in vitro and in vivo in models of Huntington's disease
J Huntingtons Dis
January 1, 2012
1 (2) :195-210
PMID: 23393546
PMCID: 3564659
Sontag EM, Lotz GP, Yang G, Sontag CJ, Cummings BJ, Glabe CG, Muchowski PJ, Thompson LM
Detection of mutant huntingtin aggregation conformers and modulation of SDS-soluble fibrillar oligomers by small molecules
J Huntingtons Dis
January 1, 2012
1 (1) :119-32
PMID: 24086178
PMCID: 3786168
Galvan L, Andre VM, Wang EA, Cepeda C, Levine MS
Functional differences between direct and indirect striatal output pathways in Huntington`s disease
J Huntingtons Dis
January 1, 2012
1 (1) :17-25
PMID: 25063187
PMCID: PMC4116084
Oakeshott S, Port R, Cummins-Sutphen J, Berger J, Watson-Johnson J, Ramboz S, Paterson N, Kwak S, Howland D, Brunner D
A mixed fixed ratio/progressive ratio procedure reveals an apathy phenotype in the BAC HD and the z_Q175 KI mouse models of Huntington`s disease
PLoS Curr
January 1, 2012
4 :e4f972cffe82c0
PMID: 23925262
PMCID: PMC3729251
Heikkinen T, Lehtimaki K, Vartiainen N, Puolivali J, Hendricks SJ, Glaser JR, Bradaia A, Wadel K, Touller C, Kontkanen O, Yrjanheikki JM, Buisson B, Howland D, Beaumont V, Munoz-Sanjuan I, Park LC
Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington`s disease
PLoS One
January 1, 2012
7 (12) :e50717
PMID: 23284644
PMCID: PMC3527436
Menalled LB, Kudwa AE, Miller S, Fitzpatrick J, Watson-Johnson J, Keating N, Ruiz M, Mushlin R, Alosio W, McConnell K, Connor D, Murphy C, Oakeshott S, Kwan M, Beltran J, Ghavami A, Brunner D, Park LC, Ramboz S, Howland D
Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington`s disease: zQ175
PLoS One
January 1, 2012
7 (12) :e49838
PMID: 23284626
PMCID: PMC3527464
Beconi M, Aziz O, Matthews K, Moumne L, O`Connell C, Yates D, Clifton S, Pett H, Vann J, Crowley L, Haughan AF, Smith DL, Woodman B, Bates GP, Brookfield F, Burli RW, McAllister G, Dominguez C, Munoz-Sanjuan I, Beaumont V
Oral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivo
PLoS One
January 1, 2012
7 (9) :e44498
PMID: 22973455
PMCID: 3433414
Tabrizi SJ, Reilmann R, Roos RAC, Durr A, Leavitt B, Owen G, Jones R, Johnson H, Craufurd D, Hicks SL, Kennard C, Landwehrmeyer B, Stout JC, Borowsky B, Scahill RI, Frost C, Langbehn DR
Potential endpoints for clinical trials in premanifest and early Huntington`s disease in the TRACK-HD study: analysis of 24 month observational data
The Lancet Neurology
January 1, 2012
11 (1) :42-53
PMID: 22137354
Quarrell OW, Handley O, O`Donovan K, Dumoulin C, Ramos-Arroyo M, Biunno I, Bauer P, Kline M, Landwehrmeyer GB, European Huntington`s Disease Network
Discrepancies in reporting the CAG repeat lengths for Huntington`s disease
Eur J Hum Genet
January 1, 2012
20 (1) :20-6
PMID: 21811303
PMCID: PMC3234505
Raymond LA, André VM, Cepeda C, Gladding CM, Milnerwood AJ, Levine MS
Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function
December 15, 2011
198 :252-73
PMID: 21907762
PMCID: 3221774
Cowin RM, Bui N, Graham D, Green JR, Grueninger S, Yuva-Paylor LA, Syed AU, Weiss A, Paylor R
Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability
PLoS One
December 7, 2011
6 (12) :e28409
PMID: 22163300
PMCID: 3233565
Zhang Y, Long JD, Mills JA, Warner JH, Lu W, Paulsen JS, PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Indexing disease progression at study entry with individuals at-risk for Huntington disease
Am J Med Genet B Neuropsychiatr Genet
December 2, 2011
156B (7) :751-63
PMID: 21858921
PMCID: 3174494
Majid DS, Aron AR, Thompson W, Sheldon S, Hamza S, Stoffers D, Holland D, Goldstein J, Corey-Bloom J, Dale AM
Basal ganglia atrophy in prodromal Huntington's disease is detectable over one year using automated segmentation
Mov Disord
December 1, 2011
26 (14) :2544-51
PMID: 21932302
PMCID: 5615846
Paulsen JS
Cognitive impairment in Huntington disease: diagnosis and treatment
Curr Neurol Neurosci Rep
October 1, 2011
11 (5) :474-83
PMID: 21861097
PMCID: 3628771
van der Burg JM, Winqvist A, Aziz NA, Maat-Schieman ML, Roos RA, Bates GP, Brundin P, Björkqvist M, Wierup N
Gastrointestinal dysfunction contributes to weight loss in Huntington's disease mice
Neurobiol Dis
October 1, 2011
44 (1) :1-8
PMID: 21624468
Benraiss A, Goldman SA
Cellular therapy and induced neuronal replacement for Huntington's disease
October 1, 2011
8 (4) :577-90
PMID: 21971961
PMCID: 3250300
Maillard MC, Brookfield FA, Courtney SM, Eustache FM, Gemkow MJ, Handel RK, Johnson LC, Johnson PD, Kerry MA, Krieger F, Meniconi M, Munoz-Sanjuan I, Palfrey JJ, Park H, Schaertl S, Taylor MG, Weddell D, Dominguez C
Exploiting differences in caspase-2 and -3 S(2) subsites for selectivity: structure-based design, solid-phase synthesis and in vitro activity of novel substrate-based caspase-2 inhibitors
Bioorg Med Chem
October 1, 2011
19 (19) :5833-51
PMID: 21903398
Muller I, Lamers MB, Ritchie AJ, Dominguez C, Munoz-Sanjuan I, Kiselyov A
Structure of human caspase-6 in complex with Z-VAD-FMK: New peptide binding mode observed for the non-canonical caspase conformation
Bioorg Med Chem Lett
September 15, 2011
21 (18) :5244-7
PMID: 21820899
Valenza M, Cattaneo E
Emerging roles for cholesterol in Huntington's disease
Trends Neurosci
September 1, 2011
34 (9) :474-86
PMID: 21774998
Huang K, Sanders SS, Kang R, Carroll JB, Sutton L, Wan J, Singaraja R, Young FB, Liu L, El-Husseini A, Davis NG, Hayden MR
Wild-type HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14
Hum Mol Genet
September 1, 2011
20 (17) :3356-65
PMID: 21636527
PMCID: PMC3153302
Carroll JB, Southwell AL, Graham RK, Lerch JP, Ehrnhoefer DE, Cao LP, Zhang WN, Deng Y, Bissada N, Henkelman RM, Hayden MR
Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease
Mol Neurodegener
August 19, 2011
6 :59
PMID: 21854568
PMCID: 3180273
Wetzel HH, Gehl CR, Dellefave-Castillo L, Schiffman JF, Shannon KM, Paulsen JS, Huntington Study Group
Suicidal ideation in Huntington disease: the role of comorbidity
Psychiatry Res
August 15, 2011
188 (3) :372-6
PMID: 21605914
PMCID: 3790459
van den Bogaard SJ, Dumas EM, Ferrarini L, Milles J, van Buchem MA, van der Grond J, Roos RA
Shape analysis of subcortical nuclei in Huntington`s disease, global versus local atrophy--results from the TRACK-HD study
J Neurol Sci
August 15, 2011
307 (43832) :60-8
PMID: 21624624
Zuccato C, Marullo M, Vitali B, Tarditi A, Mariotti C, Valenza M, Lahiri N, Wild EJ, Sassone J, Ciammola A, Bachoud-Lèvi AC, Tabrizi SJ, Di Donato S, Cattaneo E
Brain-derived neurotrophic factor in patients with Huntington's disease
PLoS One
August 12, 2011
6 (8) :e22966
PMID: 21857974
PMCID: 3155522
Williams J, Downing N, Vaccarino AL, Guttman M, Paulsen JS
Self reports of day-to-day function in a small cohort of people with prodromal and early HD
PLoS Curr
August 11, 2011
3 :RRN1254
PMID: 21901173
PMCID: 3154838
Hobbs NZ, Pedrick AV, Say MJ, Frost C, Dar Santos R, Coleman A, Sturrock A, Craufurd D, Stout JC, Leavitt BR, Barnes J, Tabrizi SJ, Scahill RI
The structural involvement of the cingulate cortex in premanifest and early Huntington`s disease
Mov Disord
August 1, 2011
26 (9) :1684-90
PMID: 21557312
Ready RE, O’Rourke JJ, Paulsen JS
Quality of life in prodromal HD: Qualitative analyses of discourse from participants and companions
Neurol Res Int
July 21, 2011
PMID: 21822491
PMCID: 3142873
Muller I, Lamers MB, Ritchie AJ, Park H, Dominguez C, Munoz-Sanjuan I, Maillard M, Kiselyov A
A new apo-caspase-6 crystal form reveals the active conformation of the apoenzyme
J Mol Biol
July 8, 2011
410 (2) :307-15
PMID: 21621544
Reinhart PH, Kaltenbach LS, Essrich C, Dunn DE, Eudailey JA, DeMarco CT, Turmel GJ, Whaley JC, Wood A, Cho S, Lo DC
Identification of anti-inflammatory targets for Huntington's disease using a brain slice-based screening assay
Neurobiol Dis
July 1, 2011
43 (1) :248-56
PMID: 21458569
PMCID: 3104027
Williams RH, Morton AJ, Burdakov D
Paradoxical function of orexin/hypocretin circuits in a mouse model of Huntington's disease
Neurobiol Dis
June 1, 2011
42 (3) :438-45
PMID: 21324360
PMCID: 5767114
O`Rourke JJ, Beglinger LJ, Smith MM, Mills J, Moser DJ, Rowe KC, Langbehn DR, Duff K, Stout JC, Harrington DL, Carlozzi N, Paulsen JS
The Trail Making Test in prodromal Huntington disease: contributions of disease progression to test performance
J Clin Exp Neuropsychol
June 1, 2011
33 (5) :567-79
PMID: 21302170
PMCID: PMC3159183
Vaccarino AL, Anderson K, Borowsky B, Duff K, Giuliano J, Guttman M, Ho AK, Orth M, Paulsen JS, Sills T, van Kammen DP, Evans KR, Predict HD and Registry Investigators Coordinators
An item response analysis of the motor and behavioral subscales of the unified Huntington`s disease rating scale in huntington disease gene expansion carriers
Mov Disord
April 1, 2011
26 (5) :877-84
PMID: 21370269
PMCID: PMC3157755
Aylward EH, Nopoulos PC, Ross CA, Langbehn DR, Pierson RK, Mills JA, Johnson HJ, Magnotta VA, Juhl AR, Paulsen JS, Predict-HD Investigators and Coordinators of Huntington Study Group
Longitudinal change in regional brain volumes in prodromal Huntington disease
J Neurol Neurosurg Psychiatry
April 1, 2011
82 (4) :405-10
PMID: 20884680
PMCID: PMC3105627
van den Bogaard SJ, Dumas EM, Acharya TP, Johnson H, Langbehn DR, Scahill RI, Tabrizi SJ, van Buchem MA, van der Grond J, Roos RA, TRACK-HD Investigator Group
Early atrophy of pallidum and accumbens nucleus in Huntington's disease
J Neurol
March 1, 2011
258 (3) :412-20
PMID: 20936300
PMCID: 3112014
Kudo T, Schroeder A, Loh DH, Kuljis D, Jordan MC, Roos KP, Colwell CS
Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease
Exp Neurol
March 1, 2011
228 (1) :80-90
PMID: 21184755
PMCID: 4346330
Duzdevich D, Li J, Whang J, Takahashi H, Takeyasu K, Dryden DT, Morton AJ, Edwardson JM
Unusual structures are present in DNA fragments containing super-long Huntingtin CAG repeats
PLoS One
February 11, 2011
6 (2) :e17119
PMID: 21347256
PMCID: 3037965