Scientific Publications

Listed here are over 600 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, 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
Yerkes S, Vesenka J, Kmiec EB
A stable G-quartet binds to a huntingtin protein fragment containing expanded polyglutamine tracks
J Neurosci Res
February 1, 2010
88 (2) :335-45
PMID: 19705452
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
Thompson LM, Aiken CT, Kaltenbach LS, Agrawal N, Illes K, Khoshnan A, Martinez-Vincente M, Arrasate M, O’Rourke JG, Khashwji H, Lukacsovich T, Zhu YZ, Lau AL, Massey A, Hayden MR, Zeitlin SO, Finkbeiner S, Green KN, LaFerla FM, Bates G, Huang L, Patterson PH, Lo DC, Cuervo AM, Marsh JL, Steffan JS
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome
J Cell Biol
December 28, 2009
187 (7) :1083-99
PMID: 20026656
PMCID: 2806289
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
Hobbs NZ, Henley SM, Wild EJ, Leung KK, Frost C, Barker RA, Scahill RI, Barnes J, Tabrizi SJ, Fox NC
Automated quantification of caudate atrophy by local registration of serial MRI: evaluation and application in Huntington's disease
Neuroimage
October 1, 2009
47 (4) :1659-65
PMID: 19523522
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
Zuccato C, Cattaneo E
Brain-derived neurotrophic factor in neurodegenerative diseases
Nat Rev Neurol
June 1, 2009
5 (6) :311-22
PMID: 19498435
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
Pallier PN, Drew CJ, Morton AJ
The detection and measurement of locomotor deficits in a transgenic mouse model of Huntington's disease are task- and protocol-dependent: influence of non-motor factors on locomotor function
Brain Res Bull
March 30, 2009
78 (6) :347-55
PMID: 19010400
Joshi PR, Wu NP, André VM, Cummings DM, Cepeda C, Joyce JA, Carroll JB, Leavitt BR, Hayden MR, Levine MS, Bamford NS
Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease
J Neurosci
February 25, 2009
29 (8) :2414-27
PMID: 19244517
PMCID: 2670193
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
Warby SC, Doty CN, Graham RK, Carroll JB, Yang YZ, Singaraja RR, Overall CM, Hayden MR
Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus
Hum Mol Genet
August 1, 2008
17 (15) :2390-404
PMID: 18445618
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
Lerch JP, Carroll JB, Spring S, Bertram LN, Schwab C, Hayden MR, Henkelman RM
Automated deformation analysis in the YAC128 Huntington disease mouse model
Neuroimage
January 1, 2008
39 (1) :32-9
PMID: 17942324
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
Heng MY, Tallaksen-Greene SJ, Detloff PJ, Albin RL
Longitudinal evaluation of the Hdh(CAG)150 knock-in murine model of Huntington's disease
J Neurosci
August 22, 2007
27 (34) :8989-98
PMID: 17715336
PMCID: 6672210
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
Voisine C, Varma H, Walker N, Bates EA, Stockwell BR, Hart AC
Identification of potential therapeutic drugs for huntington's disease using Caenorhabditis elegans
PLoS One
June 6, 2007
2 (6) :e504
PMID: 17551584
PMCID: 1876812
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
Varma H, Voisine C, DeMarco CT, Cattaneo E, Lo DC, Hart AC, Stockwell BR
Selective inhibitors of death in mutant huntingtin cells
Nat Chem Biol
February 1, 2007
3 (2) :99-100
PMID: 17195849
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
Weydt P, Pineda VV, Torrence AE, Libby RT, Satterfield TF, Lazarowski ER, Gilbert ML, Morton GJ, Bammler TK, Strand AD, Cui L, Beyer RP, Easley CN, Smith AC, Krainc D, Luquet S, Sweet IR, Schwartz MW, La Spada AR
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration
Cell Metab
November 1, 2006
4 (5) :349-62
PMID: 17055784
Cao C, Temel Y, Blokland A, Ozen H, Steinbusch HW, Vlamings R, Nguyen HP, von Hörsten S, Schmitz C, Visser-Vandewalle V
Progressive deterioration of reaction time performance and choreiform symptoms in a new Huntington's disease transgenic ratmodel
Behav Brain Res
June 30, 2006
170 (2) :257-61
PMID: 16569446