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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Harrington DL, Smith MM, Zhang Y, Carlozzi NE, Paulsen JS, PREDICT-HD Investigators of the Huntington Study Group
Cognitive domains that predict time to diagnosis in prodromal Huntington disease
J Neurol Neurosurg Psychiatry
June 1, 2012
83 (6) :612-9
PMID: 22451099
PMCID: 3812822
Giles P, Elliston L, Higgs GV, Brooks SP, Dunnett SB, Jones L
Longitudinal analysis of gene expression and behaviour in the HdhQ150 mouse model of Huntington's disease
Brain Res Bull
June 1, 2012
88 (2-3) :199-209
PMID: 22001697
Bowles KR, Brooks SP, Dunnett SB, Jones L
Gene expression and behaviour in mouse models of HD
Brain Res Bull
June 1, 2012
88 (2-3) :276-84
PMID: 21854837
Long JD, Matson WR, Juhl AR, Leavitt BR, Paulsen JS, Predict-Hd Investigators, Coordinators of the Huntington Study Group
8OHdG as a marker for Huntington disease progression
Neurobiol Dis
June 1, 2012
46 (3) :625-34
PMID: 22414782
PMCID: PMC3784019
Brooks SP, Janghra N, Workman VL, Bayram-Weston Z, Jones L, Dunnett SB
Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington`s disease transgenic mice
Brain Res Bull
June 1, 2012
88 (43864) :94-103
PMID: 21272613
Wood NI, Sawiak SJ, Buonincontri G, Niu Y, Kane AD, Carpenter TA, Giussani DA, Morton AJ
Direct evidence of progressive cardiac dysfunction in a transgenic mouse model of Huntington's disease
J Huntingtons Dis
May 31, 2012
1 (1) :57-64
PMID: 24339845
PMCID: 3856869
Gafni J, Papanikolaou T, Degiacomo F, Holcomb J, Chen S, Menalled L, Kudwa A, Fitzpatrick J, Miller S, Ramboz S, Tuunanen PI, Lehtimaki KK, Yang XW, Park L, Kwak S, Howland D, Park H, Ellerby LM
Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment
J Neurosci
May 30, 2012
32 (22) :7454-65
PMID: 22649225
PMCID: 3454486
Vidal RL, Figueroa A, Court FA, Thielen P, Molina C, Wirth C, Caballero B, Kiffin R, Segura-Aguilar J, Cuervo AM, Glimcher LH, Hetz C
Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy
Hum Mol Genet
May 15, 2012
21 (10) :2245-62
PMID: 22337954
PMCID: 3335312
Nucifora LG, Burke KA, Feng X, Arbez N, Zhu S, Miller J, Yang G, Ratovitski T, Delannoy M, Muchowski PJ, Finkbeiner S, Legleiter J, Ross CA, Poirier MA
Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein
J Biol Chem
May 4, 2012
287 (19) :16017-28
PMID: 22433867
PMCID: PMC3346083
Young FB, Butland SL, Sanders SS, Sutton LM, Hayden MR
Putting proteins in their place: palmitoylation in Huntington disease and other neuropsychiatric diseases
Prog Neurobiol
May 1, 2012
97 (2) :220-38
PMID: 22155432
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
Neuroimage
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
Neuroimage
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
Neurology
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