Scientific Publications

Listed here are over 850 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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Tabrizi SJ, Scahill RI, Durr A, Roos RA, Leavitt BR, Jones R, Landwehrmeyer GB, Fox NC, Johnson H, Hicks SL, Kennard C, Craufurd D, Frost C, Langbehn DR, Reilmann R, Stout JC, TRACK-HD Investigators
Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis
Lancet Neurol
January 1, 2011
10 (1) :31-42
PMID: 21130037
Morse RJ, Leeds JM, Macdonald D, Park L, Toledo-Sherman L, Pacifici R
Pharmaceutical development for Huntington’s disease. In: Neurobiology of Huntington's disease: Applications to drug discovery
Neurobiology of Huntington's Disease
January 1, 2011
8
PMID: 21882420
Oakeshott S, Port RG, Cummins-Sutphen J, Watson-Johnson J, Ramboz S, Park L, Howland D, Brunner D
HD mouse models reveal clear deficits in learning to perform a simple instrumental response
PLoS Curr
January 1, 2011
3 :RRN1282
PMID: 22512000
PMCID: PMC3327146
Vaccarino AL, Sills T, Anderson KE, Borowsky B, Craufurd D, Giuliano J, Goodman L, Guttman M, Kupchak P, Ho AK, Paulsen JS, Stout J, van Kammen DP, Evans K
Assessment of cognitive symptoms in prodromal and early huntington disease
PLoS Curr
January 1, 2011
3 :RRN1250
PMID: 22120841
PMCID: PMC3201666
Vaccarino AL, Sills T, Anderson KE, Endicott J, Giuliano J, Guttman M, Ho AK, Kupchak P, Paulsen JS, Warner JH, Williams J, Evans K
Assessment of day-to-day functioning in prodromal and early Huntington Disease
PLoS Curr
January 1, 2011
3 :RRN1262
PMID: 21927718
PMCID: PMC3172089
Vaccarino AL, Anonymous, Anderson KE, Borowsky B, Coccaro E, Craufurd D, Endicott J, Giuliano J, Groves M, Guttman M, Ho AK, Kupchak P, Paulsen JS, Stanford MS, van Kammen DP, Watson D, Wu KD, Evans K
Assessing behavioural manifestations prior to clinical diagnosis of huntington disease: "anger and irritability" and "obsessions and compulsions"
PLoS Curr
January 1, 2011
3 :RRN1241
PMID: 21826116
PMCID: PMC3122583
Vaccarino AL, Sills T, Anderson KE, Biglan K, Borowsky B, Giuliano J, Guttman M, Ho AK, Kennard C, Kupchak P, Landwehrmeyer GB, Michell A, Paulsen JS, Reilmann R, van Kammen DP, Warner JH, Evans K
Assessment of motor symptoms and functional impact in prodromal and early huntington disease
PLoS Curr
January 1, 2011
2 :RRN1244
PMID: 21804956
PMCID: PMC3114647
Vaccarino AL, Sills T, Anderson KE, Bachoud-Levi AC, Borowsky B, Craufurd D, Duff K, Giuliano J, Groves M, Guttman M, Kupchak P, Ho AK, Paulsen JS, Pedersen KF, van Duijn E, van Kammen DP, Evans K
Assessment of depression, anxiety and apathy in prodromal and early huntington disease
PLoS Curr
January 1, 2011
3 :RRN1242
PMID: 21731882
PMCID: PMC3124013
Fiedorowicz JG, Mills JA, Ruggle A, Langbehn D, Paulsen JS, Predict-Hd Investigators of the Huntington Study Group
Suicidal behavior in prodromal Huntington disease
Neurodegener Dis
January 1, 2011
8 (6) :483-90
PMID: 21659725
PMCID: PMC3186721
Aylward E, Mills J, Liu D, Nopoulos P, Ross CA, Pierson R, Paulsen JS
Association between Age and Striatal Volume Stratified by CAG Repeat Length in Prodromal Huntington Disease
PLoS Curr
January 1, 2011
3 :RRN1235
PMID: 21593963
PMCID: PMC3092625
Oakeshott S, Balci F, Filippov I, Murphy C, Port R, Connor D, Paintdakhi A, Lesauter J, Menalled L, Ramboz S, Kwak S, Howland D, Silver R, Brunner D
Circadian abnormalities in motor activity in a BAC transgenic mouse model of Huntington`s Disease
PLoS Curr
January 1, 2011
3 :RRN1225
PMID: 21479110
PMCID: PMC3072044
Say MJ, Jones R, Scahill RI, Dumas EM, Coleman A, Santos RC, Justo D, Campbell JC, Queller S, Shores EA, Tabrizi SJ, Stout JC, Track-Hd Investigators
Visuomotor integration deficits precede clinical onset in Huntington`s disease
Neuropsychologia
January 1, 2011
49 (2) :264-70
PMID: 21094653
Nopoulos PC, Aylward EH, Ross CA, Mills JA, Langbehn DR, Johnson HJ, Magnotta VA, Pierson RK, Beglinger LJ, Nance MA, Barker RA, Paulsen JS, Predict-Hd Investigators, Coordinators of the Huntington Study Group
Smaller intracranial volume in prodromal Huntington`s disease: evidence for abnormal neurodevelopment
Brain
January 1, 2011
134 (Pt 1) :137-42
PMID: 20923788
PMCID: PMC3025719
Stout JC, Paulsen JS, Queller S, Solomon AC, Whitlock KB, Campbell JC, Carlozzi N, Duff K, Beglinger LJ, Langbehn DR, Johnson SA, Biglan KM, Aylward EH
Neurocognitive signs in prodromal Huntington disease
Neuropsychology
January 1, 2011
25 (1) :43844
PMID: 20919768
PMCID: PMC3017660
Nopoulos PC, Aylward EH, Ross CA, Johnson HJ, Magnotta VA, Juhl AR, Pierson RK, Mills J, Langbehn DR, Paulsen JS, Predict-Hd Investigators Coordinators of Huntington Study Group
Cerebral cortex structure in prodromal Huntington disease
Neurobiol Dis
December 1, 2010
40 (3) :544-54
PMID: 20688164
PMCID: PMC2955824
Soneson C, Fontes M, Zhou Y, Denisov V, Paulsen JS, Kirik D, Petersen A, Huntington Study Group Predict-HD Investigators
Early changes in the hypothalamic region in prodromal Huntington disease revealed by MRI analysis
Neurobiol Dis
December 1, 2010
40 (3) :531-43
PMID: 20682340
PMCID: PMC2955781
Leyva MJ, Degiacomo F, Kaltenbach LS, Holcomb J, Zhang N, Gafni J, Park H, Lo DC, Salvesen GS, Ellerby LM, Ellman JA
Identification and evaluation of small molecule pan-caspase inhibitors in Huntington`s disease models
Chem Biol
November 24, 2010
17 (11) :1189-200
PMID: 21095569
PMCID: PMC3035168
Paulsen JS, Wang C, Duff K, Barker R, Nance M, Beglinger L, Moser D, Williams JK, Simpson S, Langbehn D, van Kammen DP, Predict-Hd Investigators of the Huntington Study Group
Challenges assessing clinical endpoints in early Huntington disease
Mov Disord
November 15, 2010
25 (15) :2595-603
PMID: 20623772
PMCID: PMC2978744
Graham RK, Deng Y, Carroll J, Vaid K, Cowan C, Pouladi MA, Metzler M, Bissada N, Wang L, Faull RL, Gray M, Yang XW, Raymond LA, Hayden MR
Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo
J Neurosci
November 10, 2010
30 (45) :15019-29
PMID: 21068307
PMCID: 3074336
Goodman AO, Morton AJ, Barker RA
Identifying sleep disturbances in Huntington's disease using a simple disease-focused questionnaire
PLoS Curr
October 15, 2010
2 :RRN1189
PMID: 20972477
PMCID: 2957697
Orth M, Handley OJ, Schwenke C, Dunnett SB, Craufurd D, Ho AK, Wild E, Tabrizi SJ, Landwehrmeyer GB, Investigators of the European Huntington’s Disease Network
Observing Huntington's disease: the European Huntington's Disease Network's REGISTRY
PLoS Curr
September 28, 2010
2 :RRN1184
PMID: 20890398
PMCID: 2947793
Marrakchi-Kacem L, Delmaire C, Tucholka A, Roca P, Guevara P, Poupon F, Yelnik J, Durr A, Mangin JF, Lehericy S, Poupon C
Analysis of the striato-thalamo-cortical connectivity on the cortical surface to infer biomarkers of Huntington's disease
Med Image Comput Comput Assist
September 20, 2010
13 :217-24
PMID: 20879318
Perry GM, Tallaksen-Greene S, Kumar A, Heng MY, Kneynsberg A, van Groen T, Detloff PJ, Albin RL, Lesort M
Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease
Hum Mol Genet
September 1, 2010
19 (17) :3354-71
PMID: 20558522
PMCID: 2916705
Jahanshahi A, Vlamings R, Kaya AH, Lim LW, Janssen ML, Tan S, Visser-Vandewalle V, Steinbusch HW, Temel Y
Hyperdopaminergic status in experimental Huntington disease
J Neuropathol Exp Neurol
September 1, 2010
69 (9) :910-7
PMID: 20720506
Valenza M, Leoni V, Karasinska JM, Petricca L, Fan J, Carroll J, Pouladi MA, Fossale E, Nguyen HP, Riess O, MacDonald M, Wellington C, DiDonato S, Hayden M, Cattaneo E
Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes
J Neurosci
August 11, 2010
30 (32) :10844-50
PMID: 20702713
PMCID: 3842469
Kaltenbach LS, Bolton MM, Shah B, Kanju PM, Lewis GM, Turmel GJ, Whaley JC, Trask OJ Jr, Lo DC
Composite primary neuronal high-content screening assay for Huntington's disease incorporating non-cell-autonomous interactions
J Biomol Screen
August 1, 2010
15 (7) :806-19
PMID: 20581077
Miller JP, Holcomb J, Al-Ramahi I, de Haro M, Gafni J, Zhang N, Kim E, Sanhueza M, Torcassi C, Kwak S, Botas J, Hughes RE, Ellerby LM
Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington`s disease
Neuron
July 29, 2010
67 (2) :199-212
PMID: 20670829
PMCID: PMC3098887
Maywood ES, Fraenkel E, McAllister CJ, Wood N, Reddy AB, Hastings MH, Morton AJ
Disruption of peripheral circadian timekeeping in a mouse model of Huntington's disease and its restoration by temporally scheduled feeding
J Neurosci
July 28, 2010
30 (30) :10199-204
PMID: 20668203
PMCID: 6633357
Hobbs NZ, Henley SM, Ridgway GR, Wild EJ, Barker RA, Scahill RI, Barnes J, Fox NC, Tabrizi SJ
The progression of regional atrophy in premanifest and early Huntington's disease: a longitudinal voxel-based morphometry study
J Neurol Neurosurg Psychiatry
July 1, 2010
81 (7) :756-63
PMID: 19955112
Zuccato C, Valenza M, Cattaneo E
Molecular mechanisms and potential therapeutical targets in Huntington's disease
Physiol Rev
July 1, 2010
90 (3) :905-81
PMID: 20664076
Rowe KC, Paulsen JS, Langbehn DR, Duff K, Beglinger LJ, Wang C, O’Rourke JJ, Stout JC, Moser DJ
Self-paced timing detects and tracks change in prodromal Huntington disease
Neuropsychology
July 1, 2010
24 (4) :435-42
PMID: 20604618
PMCID: 2900808
Hobbs NZ, Barnes J, Frost C, Henley SM, Wild EJ, Macdonald K, Barker RA, Scahill RI, Fox NC, Tabrizi SJ
Onset and progression of pathologic atrophy in Huntington disease: a longitudinal MR imaging study
AJNR Am J Neuroradiol
June 1, 2010
31 (6) :1036-41
PMID: 20150305
Schaertl S, Prime M, Wityak J, Dominguez C, Munoz-Sanjuan I, Pacifici RE, Courtney S, Scheel A, Macdonald D
A profiling platform for the characterization of transglutaminase 2 (TG2) inhibitors
J Biomol Screen
June 1, 2010
15 (5) :478-87
PMID: 20395409
Paulsen JS, Nopoulos PC, Aylward E, Ross CA, Johnson H, Magnotta VA, Juhl A, Pierson RK, Mills J, Langbehn D, Nance M, Predict-Hd Investigators, Coordinators of the Huntington`s Study Group
Striatal and white matter predictors of estimated diagnosis for Huntington disease
Brain Res Bull
May 31, 2010
82 (43894) :201-7
PMID: 20385209
PMCID: PMC2892238
Crittenden JR, Dunn DE, Merali FI, Woodman B, Yim M, Borkowska AE, Frosch MP, Bates GP, Housman DE, Lo DC, Graybiel AM
CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease
Hum Mol Genet
May 1, 2010
19 (9) :1756-65
PMID: 20147317
PMCID: 2850620
Becanovic K, Pouladi MA, Lim RS, Kuhn A, Pavlidis P, Luthi-Carter R, Hayden MR, Leavitt BR
Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis
Hum Mol Genet
April 15, 2010
19 (8) :1438-52
PMID: 20089533
PMCID: 2846159
Stoffers D, Sheldon S, Kuperman JM, Goldstein J, Corey-Bloom J, Aron AR
Contrasting gray and white matter changes in preclinical Huntington disease: an MRI study
Neurology
April 13, 2010
74 (15) :1208-16
PMID: 20385893
PMCID: 2872799
Menalled LB, Patry M, Ragland N, Lowden PA, Goodman J, Minnich J, Zahasky B, Park L, Leeds J, Howland D, Signer E, Tobin AJ, Brunner D
Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline
PLoS One
March 22, 2010
5 (3) :e9793
PMID: 20339553
PMCID: 2842438
Langbehn DR, Hayden MR, Paulsen JS, Predict-Hd Investigators of the Huntington Study Group
CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches
Am J Med Genet B Neuropsychiatr Genet
March 5, 2010
153B (2) :397-408
PMID: 19548255
PMCID: PMC3048807
Wood NI, Carta V, Milde S, Skillings EA, McAllister CJ, Ang YL, Duguid A, Wijesuriya N, Afzal SM, Fernandes JX, Leong TW, Morton AJ
Responses to environmental enrichment differ with sex and genotype in a transgenic mouse model of Huntington's disease
PLoS One
February 12, 2010
5 (2) :e9077
PMID: 20174443
PMCID: 2820540