Scientific Publications

Listed here are over 500 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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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
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
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
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
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
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
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
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
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
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
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
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
Paulsen JS
Early detection of Huntington disease
Future Neurol
January 1, 2010
5 (1)
PMID: 24348095
PMCID: PMC3860286
Bechtel N, Scahill RI, Rosas HD, Acharya T, van den Bogaard SJ, Jauffret C, Say MJ, Sturrock A, Johnson H, Onorato CE, Salat DH, Durr A, Leavitt BR, Roos RA, Landwehrmeyer GB, Langbehn DR, Stout JC, Tabrizi SJ, Reilmann R
Tapping linked to function and structure in premanifest and symptomatic Huntington disease
Neurology
January 1, 2010
75 (24) :2150-60
PMID: 21068430
PMCID: PMC3013584