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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Ritch JJ, Valencia A, Alexander J, Sapp E, Gatune L, Sangrey GR, Sinha S, Scherber CM, Zeitlin S, Sadri-Vakili G, Irimia D, Difiglia M, Kegel KB
Multiple phenotypes in Huntington disease mouse neural stem cells
Mol Cell Neurosci
May 1, 2012
50 (1) :70-81
PMID: 22508027
PMCID: 3383872
Aggarwal M, Duan W, Hou Z, Rakesh N, Peng Q, Ross CA, Miller MI, Mori S, Zhang J
Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging
May 1, 2012
60 (4) :2086-95
PMID: 22342677
PMCID: 3321064
van den Bogaard SJ, Dumas EM, Milles J, Reilmann R, Stout JC, Craufurd D, van Buchem MA, van der Grond J, Roos RA
Magnetization transfer imaging in premanifest and manifest Huntington disease
AJNR Am J Neuroradiol
May 1, 2012
33 (5) :884-9
PMID: 22241387
PMCID: 7968816
Uribe V, Wong BK, Graham RK, Cusack CL, Skotte NH, Pouladi MA, Xie Y, Feinberg K, Ou Y, Ouyang Y, Deng Y, Franciosi S, Bissada N, Spreeuw A, Zhang W, Ehrnhoefer DE, Vaid K, Miller FD, Deshmukh M, Howland D, Hayden MR
Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice
Hum Mol Genet
May 1, 2012
21 (9) :1954-67
PMID: 22262731
PMCID: PMC3315204
Aylward EH, Liu D, Nopoulos PC, Ross CA, Pierson RK, Mills JA, Long JD, Paulsen JS, Predict-Hd Investigators, Coordinators of the Huntington Study Group
Striatal volume contributes to the prediction of onset of Huntington disease in incident cases
Biol Psychiatry
May 1, 2012
71 (9) :822-8
PMID: 21907324
PMCID: PMC3237730
Rossin F, D`Eletto M, Macdonald D, Farrace MG, Piacentini M
TG2 transamidating activity acts as a reostat controlling the interplay between apoptosis and autophagy
Amino Acids
May 1, 2012
42 (5) :1793-802
PMID: 21479826
Rowe KC, Paulsen JS, Langbehn DR, Wang C, Mills J, Beglinger LJ, Smith MM, Epping EA, Fiedorowicz JG, Duff K, Ruggle A, Moser DJ, Predict-Hd Investigators of the Huntington Study Group
Patterns of serotonergic antidepressant usage in prodromal Huntington disease
Psychiatry Res
April 30, 2012
196 (43864) :309-14
PMID: 22397915
PMCID: PMC3763706
Fonteijn HM, Modat M, Clarkson MJ, Barnes J, Lehmann M, Hobbs NZ, Scahill RI, Tabrizi SJ, Ourselin S, Fox NC, Alexander DC
An event-based model for disease progression and its application in familial Alzheimer's disease and Huntington's disease
April 15, 2012
60 (3) :1880-9
PMID: 22281676
Camnasio S, Delli Carri A, Lombardo A, Grad I, Mariotti C, Castucci A, Rozell B, Lo Riso P, Castiglioni V, Zuccato C, Rochon C, Takashima Y, Diaferia G, Biunno I, Gellera C, Jaconi M, Smith A, Hovatta O, Naldini L, Di Donato S, Feki A, Cattaneo E
The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington's disease patients demonstrates mutation related enhanced lysosomal activity
Neurobiol Dis
April 1, 2012
46 (1) :41-51
PMID: 22405424
Vellas B, Hampel H, Rouge-Bugat ME, Grundman M, Andrieu S, Abu-Shakra S, Bateman R, Berman R, Black R, Carrillo M, Donohue M, Mintun M, Morris J, Petersen R, Thomas RG, Suhy J, Schneider L, Seely L, Tariot P, Touchon J, Weiner M, Sampaio C, Aisen P, Task Force Participants
Alzheimer`s disease therapeutic trials: EU/US Task Force report on recruitment, retention, and methodology
J Nutr Health Aging
April 1, 2012
16 (4) :339-45
PMID: 22499454
Lee JM, Ramos EM, Lee JH, Gillis T, Mysore JS, Hayden MR, Warby SC, Morrison P, Nance M, Ross CA, Margolis RL, Squitieri F, Orobello S, Di Donato S, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Abramson RK, Marder K, Sequeiros J, Paulsen JS, PREDICT-HD study of the Huntington Study Group, Landwehrmeyer GB, REGISTRY study of EHDN, Myers RH, HD-MAPS Study Group, MacDonald ME, Gusella JF, COHORT study of the HSG
CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion
March 6, 2012
78 (10) :690-5
PMID: 22323755
PMCID: 3306163
Kraft AD, Kaltenbach LS, Lo DC, Harry GJ
Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons
Neurobiol Aging
March 1, 2012
33 (3)
PMID: 21482444
PMCID: 3135676
Baldo B, Paganetti P, Grueninger S, Marcellin D, Kaltenbach LS, Lo DC, Semmelroth M, Zivanovic A, Abramowski D, Smith D, Lotz GP, Bates GP, Weiss A
TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease
Chem Biol
February 24, 2012
19 (2) :264-75
PMID: 22365609
Dorsey E, Huntington Study Group COHORT Investigators
Characterization of a large group of individuals with Huntington disease and their relatives enrolled in the COHORT study
PLoS One
February 16, 2012
7 (2) :e29522
PMID: 22359536
PMCID: 3281013
Beconi MG, Howland D, Park L, Lyons K, Giuliano J, Dominguez C, Munoz-Sanjuan I, Pacifici R
Pharmacokinetics of memantine in rats and mice
PLoS Curr
February 15, 2012
3 :RRN1291
PMID: 22307216
PMCID: PMC3269340
Menalled L, El-Khodor BF, Hornberger M, Park L, Howland D, Brunner D
Effect of the rd1 mutation on motor performance in R6/2 and wild type mice
PLoS Curr
February 13, 2012
4 :RRN1303
PMID: 22367489
PMCID: 3278853
Prime ME, Andersen OA, Barker JJ, Brooks MA, Cheng RK, Toogood-Johnson I, Courtney SM, Brookfield FA, Yarnold CJ, Marston RW, Johnson PD, Johnsen SF, Palfrey JJ, Vaidya D, Erfan S, Ichihara O, Felicetti B, Palan S, Pedret-Dunn A, Schaertl S, Sternberger I, Ebneth A, Scheel A, Winkler D, Toledo-Sherman L, Beconi M, Macdonald D, Munoz-Sanjuan I, Dominguez C, Wityak J
Discovery and structure-activity relationship of potent and selective covalent inhibitors of transglutaminase 2 for Huntington`s disease
J Med Chem
February 9, 2012
55 (3) :1021-46
PMID: 22224594
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