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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Baxa M, Hruska-Plochan M, Juhas S, Vodicka P, Pavlok A, Juhasova J, Miyanohara A, Nejime T, Klima J, Macakova M, Marsala S, Weiss A, Kubickova S, Musilova P, Vrtel R, Sontag EM, Thompson LM, Schier J, Hansikova H, Howland DS, Cattaneo E, DiFiglia M, Marsala M, Motlik J
A transgenic minipig model of Huntington`s Disease
J Huntingtons Dis
January 1, 2013
2 (1) :47-68
PMID: 25063429
Morton AJ, Howland DS
Large genetic animal models of Huntington`s Disease
J Huntingtons Dis
January 1, 2013
2 (1) :43909
PMID: 25063426
Liu W, Chaurette J, Pfister EL, Kennington LA, Chase KO, Bullock J, Vonsattel JP, Faull RL, Macdonald D, DiFiglia M, Zamore PD, Aronin N
Increased steady-state mutant huntingtin mRNA in Huntington`s Disease brain
J Huntingtons Dis
January 1, 2013
2 (4) :491-500
PMID: 25062733
Valencia A, Sapp E, Kimm JS, McClory H, Ansong KA, Yohrling G, Kwak S, Kegel KB, Green KM, Shaffer SA, Aronin N, DiFiglia M
Striatal synaptosomes from Hdh140Q/140Q knock-in mice have altered protein levels, novel sites of methionine oxidation, and excess glutamate release after stimulation
J Huntingtons Dis
January 1, 2013
2 (4) :459-75
PMID: 24696705
PMCID: PMC3970728
Oakeshott S, Farrar A, Port R, Cummins-Sutphen J, Berger J, Watson-Johnson J, Ramboz S, Howland D, Brunner D
Deficits in a simple visual go/no-go discrimination task in two mouse models of Huntington`s Disease
PLoS Curr
January 1, 2013
5
PMID: 24270512
PMCID: PMC3828223
Kudwa AE, Menalled LB, Oakeshott S, Murphy C, Mushlin R, Fitzpatrick J, Miller SF, McConnell K, Port R, Torello J, Howland D, Ramboz S, Brunner D
Increased body weight of the BAC HD transgenic mouse model of Huntington`s Disease accounts for some but not all of the observed HD-like motor deficits
PLoS Curr
January 1, 2013
5
PMID: 24042107
PMCID: PMC3770835
Balci F, Oakeshott S, Shamy JL, El-Khodor BF, Filippov I, Mushlin R, Port R, Connor D, Paintdakhi A, Menalled L, Ramboz S, Howland D, Kwak S, Brunner D
High-throughput automated phenotyping of two genetic mouse models of Huntington`s Disease
PLoS Curr
January 1, 2013
5
PMID: 23863947
PMCID: PMC3710674
Gray MA, Egan GF, Ando A, Churchyard A, Chua P, Stout JC, Georgiou-Karistianis N
Prefrontal activity in Huntington`s disease reflects cognitive and neuropsychiatric disturbances: the IMAGE-HD study
Exp Neurol
January 1, 2013
239 :218-28
PMID: 23123406
Sawiak SJ, Wood NI, Carpenter TA, Morton AJ
Huntington's disease mouse models online: high-resolution MRI images with stereotaxic templates for computational neuroanatomy
PLoS One
December 31, 2012
7 (12) :e53361
PMID: 23300918
PMCID: 3534048
Bouchard J, Truong J, Bouchard K, Dunkelberger D, Desrayaud S, Moussaoui S, Tabrizi SJ, Stella N, Muchowski PJ
Cannabinoid receptor 2 signaling in peripheral immune cells modulates disease onset and severity in mouse models of Huntington's disease
J Neurosci
December 12, 2012
32 (50) :18259-68
PMID: 23238740
PMCID: 3753072
Vlamings R, Benazzouz A, Chetrit J, Janssen ML, Kozan R, Visser-Vandewalle V, Steinbusch HW, von Hörsten S, Temel Y
Metabolic and electrophysiological changes in the basal ganglia of transgenic Huntington's disease rats
Neurobiol Dis
December 3, 2012
48 (3) :488-94
PMID: 22813864
Beconi MG, Yates D, Lyons K, Matthews K, Clifton S, Mead T, Prime M, Winkler D, O`Connell C, Walter D, Toledo-Sherman L, Munoz-Sanjuan I, Dominguez C
Metabolism and pharmacokinetics of JM6 in mice: JM6 is not a prodrug for Ro-61-8048
Drug Metab Dispos
December 1, 2012
40 (12) :2297-306
PMID: 22942319
Miller JP, Yates BE, Al-Ramahi I, Berman AE, Sanhueza M, Kim E, de Haro M, DeGiacomo F, Torcassi C, Holcomb J, Gafni J, Mooney SD, Botas J, Ellerby LM, Hughes RE
A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease
PLoS Genet
November 29, 2012
8 (11) :e1003042
PMID: 23209424
PMCID: 3510027
Hadzi TC, Hendricks AE, Latourelle JC, Lunetta KL, Cupples LA, Gillis T, Mysore JS, Gusella JF, MacDonald ME, Myers RH, Vonsattel JP
Assessment of cortical and striatal involvement in 523 Huntington disease brains
Neurology
October 16, 2012
79 (16) :1708-15
PMID: 23035064
PMCID: 3468776
Marullo M, Valenza M, Leoni V, Caccia C, Scarlatti C, De Mario A, Zuccato C, Di Donato S, Carafoli E, Cattaneo E
Pitfalls in the detection of cholesterol in Huntington's disease models
PLoS Curr
October 11, 2012
4 :e505886e9a1968
PMID: 23145355
PMCID: 3493072
Weiss A, Trager U, Wild EJ, Grueninger S, Farmer R, Landles C, Scahill RI, Lahiri N, Haider S, Macdonald D, Frost C, Bates GP, Bilbe G, Kuhn R, Andre R, Tabrizi SJ
Mutant huntingtin fragmentation in immune cells tracks Huntington`s disease progression
J Clin Invest
October 1, 2012
122 (10) :3731-6
PMID: 22996692
PMCID: PMC3461928
Reiner A, Wang HB, Del Mar N, Sakata K, Yoo W, Deng YP
BDNF may play a differential role in the protective effect of the mGluR2/3 agonist LY379268 on striatal projection neurons in R6/2 Huntington's disease mice
Brain Res
September 14, 2012
1473 :161-72
PMID: 22820300
PMCID: PMC3428435
Prime ME, Brookfield FA, Courtney SM, Gaines S, Marston RW, Ichihara O, Li M, Vaidya D, Williams H, Pedret-Dunn A, Reed L, Schaertl S, Toledo-Sherman L, Beconi M, Macdonald D, Munoz-Sanjuan I, Dominguez C, Wityak J
Irreversible 4-Aminopiperidine Transglutaminase 2 Inhibitors for Huntington`s Disease
ACS Med Chem Lett
September 13, 2012
3 (9) :731-5
PMID: 24900540
PMCID: PMC4025765
Parker JA, Vazquez-Manrique RP, Tourette C, Farina F, Offner N, Mukhopadhyay A, Orfila AM, Darbois A, Menet S, Tissenbaum HA, Neri C
Integration of β-catenin, sirtuin, and FOXO signaling protects from mutant huntingtin toxicity
J Neurosci
September 5, 2012
32 (36) :12630-40
PMID: 22956852
PMCID: 3780431
Smith MM, Mills JA, Epping EA, Westervelt HJ, Paulsen JS, PREDICT-HD Investigators of the Huntington Study Group
Depressive symptom severity is related to poorer cognitive performance in prodromal Huntington disease
Neuropsychology
September 1, 2012
6 (5) :664-9
PMID: 22846033
PMCID: 3806339
Scahill RI, Wild EJ, Tabrizi SJ
Biomarkers for Huntington`s disease: an update
Expert Opin Med Diagn
September 1, 2012
6 (5) :371-5
PMID: 23480802
Wolf RC, Sambataro F, Vasic N, Wolf ND, Thomann PA, Saft C, Landwehrmeyer GB, Orth M
Default-mode network changes in preclinical Huntington`s disease
Exp Neurol
September 1, 2012
237 (1) :191-8
PMID: 22742947
Caron NS, Munsie LN, Keillor JW, Truant R
Using FLIM-FRET to measure conformational changes of transglutaminase type 2 in live cells
PLoS One
August 31, 2012
7 (8) :e44159
PMID: 22952912
PMCID: 3432096
Yu D, Pendergraff H, Liu J, Kordasiewicz HB, Cleveland DW, Swayze EE, Lima WF, Crooke ST, Prakash TP, Corey DR
Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression
Cell
August 31, 2012
150 (5) :895-908
PMID: 22939619
PMCID: 3444165
Sontag EM, Lotz GP, Agrawal N, Tran A, Aron R, Yang G, Necula M, Lau A, Finkbeiner S, Glabe C, Marsh JL, Muchowski PJ, Thompson LM
Methylene blue modulates huntingtin aggregation intermediates and is protective in Huntington's disease models
J Neurosci
August 8, 2012
32 (32) :11109-19
PMID: 22875942
PMCID: 3546821
HD iPSC Consortium
Induced pluripotent stem cells from patients with Huntington`s disease show CAG-repeat-expansion-associated phenotypes
Cell Stem Cell
August 3, 2012
11 (2) :264-78
PMID: 22748968
Vlamings R, Zeef DH, Janssen ML, Oosterloo M, Schaper F, Jahanshahi A, Temel Y
Lessons learned from the transgenic Huntington's disease rats
Neural Plast
July 18, 2012
2012 :682712
PMID: 22852099
PMCID: 3407652
Landles C, Weiss A, Franklin S, Howland D, Bates G
Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington's disease
PLoS Curr
July 16, 2012
4 :e4fd085bfc9973
PMID: 22919566
PMCID: 3423312
Shirasaki DI, Greiner ER, Al-Ramahi I, Gray M, Boontheung P, Geschwind DH, Botas J, Coppola G, Horvath S, Loo JA, Yang XW
Network organization of the huntingtin proteomic interactome in mammalian brain
Neuron
July 12, 2012
75 (1) :41-57
PMID: 22794259
PMCID: 3432264
Dumas EM, Versluis MJ, van den Bogaard SJ, van Osch MJ, Hart EP, van Roon-Mom WM, van Buchem MA, Webb AG, van der Grond J, Roos RA, Track-Hd investigators
Elevated brain iron is independent from atrophy in Huntington`s Disease
Neuroimage
July 2, 2012
61 (3) :558-64
PMID: 22480728
Stout JC, Jones R, Labuschagne I, O’Regan AM, Say MJ, Dumas EM, Queller S, Justo D, Santos RD, Coleman A, Hart EP, Dürr A, Leavitt BR, Roos RA, Langbehn DR, Tabrizi SJ, Frost C
Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease
J Neurol Neurosurg Psychiatry
July 1, 2012
83 (7) :687-94
PMID: 22566599
PMCID: 3368487
Reiner A, Lafferty DC, Wang HB, Del Mar N, Deng YP
The group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease mice
Neurobiol Dis
July 1, 2012
47 (1) :75-91
PMID: 22472187
PMCID: PMC3376646
Culver BP, Savas JN, Park SK, Choi JH, Zheng S, Zeitlin SO, Yates JR 3rd, Tanese N
Proteomic analysis of wild-type and mutant huntingtin-associated proteins in mouse brains identifies unique interactions and involvement in protein synthesis
J Biol Chem
June 22, 2012
287 (26) :21599-614
PMID: 22556411
PMCID: 3381125
Kordasiewicz HB, Stanek LM, Wancewicz EV, Mazur C, McAlonis MM, Pytel KA, Artates JW, Weiss A, Cheng SH, Shihabuddin LS, Hung G, Bennett CF, Cleveland DW
Sustained therapeutic reversal of Huntington`s disease by transient repression of huntingtin synthesis
Neuron
June 21, 2012
74 (6) :1031-44
PMID: 22726834
PMCID: PMC3383626
Harrington DL, Smith MM, Zhang Y, Carlozzi NE, Paulsen JS, PREDICT-HD Investigators of the Huntington Study Group
Cognitive domains that predict time to diagnosis in prodromal Huntington disease
J Neurol Neurosurg Psychiatry
June 1, 2012
83 (6) :612-9
PMID: 22451099
PMCID: 3812822
Giles P, Elliston L, Higgs GV, Brooks SP, Dunnett SB, Jones L
Longitudinal analysis of gene expression and behaviour in the HdhQ150 mouse model of Huntington's disease
Brain Res Bull
June 1, 2012
88 (2-3) :199-209
PMID: 22001697
Bowles KR, Brooks SP, Dunnett SB, Jones L
Gene expression and behaviour in mouse models of HD
Brain Res Bull
June 1, 2012
88 (2-3) :276-84
PMID: 21854837
Long JD, Matson WR, Juhl AR, Leavitt BR, Paulsen JS, Predict-Hd Investigators, Coordinators of the Huntington Study Group
8OHdG as a marker for Huntington disease progression
Neurobiol Dis
June 1, 2012
46 (3) :625-34
PMID: 22414782
PMCID: PMC3784019
Brooks SP, Janghra N, Workman VL, Bayram-Weston Z, Jones L, Dunnett SB
Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington`s disease transgenic mice
Brain Res Bull
June 1, 2012
88 (43864) :94-103
PMID: 21272613
Wood NI, Sawiak SJ, Buonincontri G, Niu Y, Kane AD, Carpenter TA, Giussani DA, Morton AJ
Direct evidence of progressive cardiac dysfunction in a transgenic mouse model of Huntington's disease
J Huntingtons Dis
May 31, 2012
1 (1) :57-64
PMID: 24339845
PMCID: 3856869