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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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
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
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
Sturrock A, Laule C, Decolongon J, Dar Santos R, Coleman AJ, Creighton S, Bechtel N, Reilmann R, Hayden MR, Tabrizi SJ, Mackay AL, Leavitt BR
Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease
Neurology
January 1, 2010
75 (19) :1702-10
PMID: 21060093
PMCID: PMC3033605
Duff K, Paulsen J, Mills J, Beglinger LJ, Moser DJ, Smith MM, Langbehn D, Stout J, Queller S, Harrington DL, PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Mild cognitive impairment in prediagnosed Huntington disease
Neurology
January 1, 2010
75 (6) :500-7
PMID: 20610833
PMCID: PMC2918475
Duff K, Paulsen JS, Beglinger LJ, Langbehn DR, Wang C, Stout JC, Ross CA, Aylward E, Carlozzi NE, Queller S, Predict-HD Investigators of the Huntington Study Group
"Frontal" behaviors before the diagnosis of Huntington`s disease and their relationship to markers of disease progression: evidence of early lack of awareness
J Neuropsychiatry Clin Neurosci
January 1, 2010
22 (2) :196-207
PMID: 20463114
PMCID: PMC2871328
Gu X, Greiner ER, Mishra R, Kodali R, Osmand A, Finkbeiner S, Steffan JS, Thompson LM, Wetzel R, Yang XW
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice
Neuron
December 24, 2009
64 (6) :828-40
PMID: 20064390
PMCID: PMC2807408
Biglan KM, Ross CA, Langbehn DR, Aylward EH, Stout JC, Queller S, Carlozzi NE, Duff K, Beglinger LJ, Paulsen JS, Predict-Hd Investigators of the Huntington Study Group
Motor abnormalities in premanifest persons with Huntington`s disease: the PREDICT-HD study
Mov Disord
September 15, 2009
24 (12) :1763-72
PMID: 19562761
PMCID: PMC3048804
Tabrizi SJ, Langbehn DR, Leavitt BR, Roos RAC, Durr A, Craufurd D, Kennard C, Hicks SL, Fox NC, Scahill RI, Borowsky B, Tobin A J, Rosas HD, Johnson H, Reilmann R, Landwehrmeyer B, Stout JC
Biological and clinical manifestations of Huntington`s disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data
The Lancet Neurology
September 1, 2009
8 (9) :791-801
PMID: 19646924
PMCID: PMC3725974
Menalled L, El-Khodor BF, Patry M, Suarez-Farinas M, Orenstein SJ, Zahasky B, Leahy C, Wheeler V, Yang XW, MacDonald M, Morton AJ, Bates G, Leeds J, Park L, Howland D, Signer E, Tobin A, Brunner D
Systematic behavioral evaluation of Huntington`s disease transgenic and knock-in mouse models
Neurobiol Dis
September 1, 2009
35 (3) :319-36
PMID: 19464370
PMCID: PMC2728344
Liu Y, Lacson R, Cassaday J, Ross DA, Kreamer A, Hudak E, Peltier R, McLaren D, Munoz-Sanjuan I, Santini F, Strulovici B, Ferrer M
Identification of small-molecule modulators of mouse SVZ progenitor cell proliferation and differentiation through high-throughput screening
J Biomol Screen
April 1, 2009
14 (4) :319-29
PMID: 19403915
Weaver KE, Richards TL, Liang O, Laurino MY, Samii A, Aylward EH
Longitudinal diffusion tensor imaging in Huntington`s Disease
Exp Neurol
April 1, 2009
216 (2) :525-9
PMID: 19320010
Munoz-Sanjuan, I
Glial progenitor cell transplantation and the generation of chimeric animal models with human brain cells: implications for novel therapeutics
Expert Opin Ther Pat
January 1, 2009
19 (12) :1639-46
PMID: 19939186
Kloppel S, Chu C, Tan GC, Draganski B, Johnson H, Paulsen JS, Kienzle W, Tabrizi SJ, Ashburner J, Frackowiak RS, PREDICT-HD Investigators of the Huntington Study Group
Automatic detection of preclinical neurodegeneration: presymptomatic Huntington disease
Neurology
January 1, 2009
72 (5) :426-31
PMID: 19188573
PMCID: PMC2817939
Southwell AL, Khoshnan A, Dunn DE, Bugg CW, Lo DC, Patterson PH
Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity
J Neurosci
September 3, 2008
28 (36) :9013-20
PMID: 18768695
PMCID: 2633448
Colin E, Zala D, Liot G, Rangone H, Borrell-Pagès M, Li XJ, Saudou F, Humbert S
Huntingtin phosphorylation acts as a molecular switch for anterograde retrograde transport in neurons
EMBO J
August 6, 2008
27 (15) :2124-34
PMID: 18615096
PMCID: 2516882
Cho SJ, Sun Y
Visual exploration of structure-activity relationship using maximum common framework
J Comput Aided Mol Des
August 1, 2008
22 (8) :571-8
PMID: 18338222
Paulsen JS, Langbehn DR, Stout JC, Aylward E, Ross CA, Nance M, Guttman M, Johnson S, MacDonald M, Beglinger LJ, Duff K, Kayson E, Biglan K, Shoulson I, Oakes D, Hayden M, Predict-HD Investigators, Coordinators of the Huntington Study Group
Detection of Huntington`s disease decades before diagnosis: the Predict-HD study
J Neurol Neurosurg Psychiatry
August 1, 2008
79 (8) :874-80
PMID: 18096682
PMCID: PMC2569211
Ciamei A, Morton AJ
Rigidity in social and emotional memory in the R6/2 mouse model of Huntington`s disease
Neurobiol Learn Mem
May 1, 2008
89 (4) :533-44
PMID: 18069020
Beglinger LJ, Paulsen JS, Watson DB, Wang C, Duff K, Langbehn DR, Moser DJ, Paulson HL, Aylward EH, Carlozzi NE, Queller S, Stout JC
Obsessive and compulsive symptoms in prediagnosed Huntington`s disease
J Clin Psychiatry
January 1, 2008
69 (11) :1758-65
PMID: 19012814
Duff K, Paulsen JS, Beglinger LJ, Langbehn DR, Stout JC, Predict-HD Investigators of the Huntington Study Group
Psychiatric symptoms in Huntington`s disease before diagnosis: the predict-HD study
Biol Psychiatry
December 15, 2007
62 (12) :1341-6
PMID: 17481592
DiFiglia M, Sena-Esteves M, Chase K, Sapp E, Pfister E, Sass M, Yoder J, Reeves P, Pandey RK, Rajeev KG, Manoharan M, Sah DW, Zamore PD, Aronin N
Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits
Proc Natl Acad Sci USA
October 23, 2007
104 (43) :17204-9
PMID: 17940007
PMCID: PMC2040405
Johnson SA, Stout JC, Solomon AC, Langbehn DR, Aylward EH, Cruce CB, Ross CA, Nance M, Kayson E, Julian-Baros E, Hayden MR, Kieburtz K, Guttman M, Oakes D, Shoulson I, Beglinger L, Duff K, Penziner E, Paulsen JS, Predict-HD Investigators of the Huntington Study Group
Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington`s disease
Brain
July 1, 2007
130 (Pt 7) :1732-44
PMID: 17584778
Solomon AC, Stout JC, Johnson SA, Langbehn DR, Aylward EH, Brandt J, Ross CA, Beglinger L, Hayden MR, Kieburtz K, Kayson E, Julian-Baros E, Duff K, Guttman M, Nance M, Oakes D, Shoulson I, Penziner E, Paulsen JS, Predict-HD Investigators of the Huntington Study Group
Verbal episodic memory declines prior to diagnosis in Huntington`s disease
Neuropsychologia
April 9, 2007
45 (8) :1767-76
PMID: 17303196
PMCID: PMC2570315
Beglinger LJ, Langbehn DR, Duff K, Stierman L, Black DW, Nehl C, Anderson K, Penziner E, Paulsen JS, Huntington Study Group Investigators
Probability of obsessive and compulsive symptoms in Huntington`s disease
Biol Psychiatry
February 1, 2007
61 (3) :415-8
PMID: 16839521
Van Raamsdonk JM, Gibson WT, Pearson J, Murphy Z, Lu G, Leavitt BR, Hayden MR
Body weight is modulated by levels of full-length huntingtin
Hum Mol Genet
May 1, 2006
15 (9) :1513-23
PMID: 16571604
Paulsen JS, Hayden M, Stout JC, Langbehn DR, Aylward E, Ross CA, Guttman M, Nance M, Kieburtz K, Oakes D, Shoulson I, Kayson E, Johnson S, Penziner E, Predict-HD Investigators of the Huntington Study Group
Preparing for preventive clinical trials: the Predict-HD study
Arch Neurol
January 1, 2006
63 (6) :883-90
PMID: 16769871
Paulsen JS, Zimbelman JL, Hinton SC, Langbehn DR, Leveroni CL, Benjamin ML, Reynolds NC, Rao SM
fMRI biomarker of early neuronal dysfunction in presymptomatic Huntington's disease
AJNR Am J Neuroradiol
November 2, 2004
25 (10) :1715-21
PMID: 15569736