Scientific Publications

Listed here are over 1000 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 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
Silvestroni A, Faull RL, Strand AD, Möller T
Distinct neuroinflammatory profile in post-mortem human Huntington's disease
Neuroreport
August 5, 2009
20 (12) :1098-103
PMID: 19590393
Pallier PN, Morton AJ
Management of sleep/wake cycles improves cognitive function in a transgenic mouse model of Huntington's disease
Brain Res
July 7, 2009
1279 :90-8
PMID: 19450569
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
Morton AJ, Glynn D, Leavens W, Zheng Z, Faull RL, Skepper JN, Wight JM
Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice
Neurobiol Dis
March 1, 2009
33 (3) :331-41
PMID: 19130884
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
Sawiak SJ, Wood NI, Williams GB, Morton AJ, Carpenter TA
Use of magnetic resonance imaging for anatomical phenotyping of the R6/2 mouse model of Huntington's diseas
Neurobiol Dis
January 1, 2009
33 (1) :12-9
PMID: 18930823
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
Henley SM, Wild EJ, Hobbs NZ, Warren JD, Frost C, Scahill RI, Ridgway GR, MacManus DG, Barker RA, Fox NC, Tabrizi SJ
Defective emotion recognition in early HD is neuropsychologically and anatomically generic
Neuropsychologia
July 1, 2008
46 (8) :2152-60
PMID: 18407301
Lerch JP, Carroll JB, Dorr A, Spring S, Evans AC, Hayden MR, Sled JG, Henkelman RM
Cortical thickness measured from MRI in the YAC128 mouse model of Huntington's disease
Neuroimage
June 1, 2008
41 (2) :243-51
PMID: 18387826
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
Anne SL, Saudou F, Humbert S
Phosphorylation of huntingtin by cyclin-dependent kinase 5 is induced by DNA damage and regulates wild-type and mutant huntingtin toxicity in neurons
J Neurosci
July 4, 2007
27 (27) :7318-28
PMID: 17611284
PMCID: 6794597
Dalrymple A, Wild EJ, Joubert R, Sathasivam K, Björkqvist M, Petersén A, Jackson GS, Isaacs JD, Kristiansen M, Bates GP, Leavitt BR, Keir G, Ward M, Tabrizi SJ
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates
J Proteome Res
July 1, 2007
6 (7) :2833-40
PMID: 17552550
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
Kung VW, Hassam R, Morton AJ, Jones S
Dopamine-dependent long term potentiation in the dorsal striatum is reduced in the R6/2 mouse model of Huntington's disease
Neuroscience
June 8, 2007
146 (4) :1571-80
PMID: 17478055
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
Kaltenbach LS, Romero E, Becklin RR, Chettier R, Bell R, Phansalkar A, Strand A, Torcassi C, Savage J, Hurlburt A, Cha GH, Ukani L, Chepanoske CL, Zhen Y, Sahasrabudhe S, Olson J, Kurschner C, Ellerby LM, Peltier JM, Botas J, Hughes RE
Huntingtin interacting proteins are genetic modifiers of neurodegeneration
PLoS Genet
May 11, 2007
3 (5) :e82
PMID: 17500595
PMCID: 1866352
Woodman B, Butler R, Landles C, Lupton MK, Tse J, Hockly E, Moffitt H, Sathasivam K, Bates GP
The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes
Brain Res Bull
April 30, 2007
72 (2-3) :83-97
PMID: 17352931
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
Guidetti P, Bates GP, Graham RK, Hayden MR, Leavitt BR, MacDonald ME, Slow EJ, Wheeler VC, Woodman B, Schwarcz R
Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice
Neurobiol Dis
July 1, 2006
23 (1) :190-7
PMID: 16697652
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
Lloret A, Dragileva E, Teed A, Espinola J, Fossale E, Gillis T, Lopez E, Myers RH, MacDonald ME, Wheeler VC
Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice
Hum Mol Genet
June 15, 2006
15 (12) :2015-24
PMID: 16687439
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