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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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
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
Sah DW, Aronin N
Oligonucleotide therapeutic approaches for Huntington disease
J Clin Invest
February 1, 2011
121 (2) :500-7
PMID: 21285523
PMCID: 3026739
Eidelberg D, Surmeier DJ
Brain networks in Huntington disease
J Clin Invest
February 1, 2011
121 (2) :484-92
PMID: 21285521
PMCID: 3026742
Munoz-Sanjuan I, Bates GP
The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease
J Clin Invest
February 1, 2011
121 (2) :476-83
PMID: 21285520
PMCID: PMC3026740
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