CHDI
Listed here are over 950 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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Title
Journal
Rattray I, Smith E, Gale R, Matsumoto K, Bates GP, Modo M
Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HD
Lewandowski NM, Bordelon Y, Brickman AM, Angulo S, Khan U, Muraskin J, Griffith EY, Wasserman P, Menalled L, Vonsattel JP, Marder K, Small SA, Moreno H
Regional vulnerability in Huntington's disease: fMRI-guided molecular analysis in patients and a mouse model of disease
Biglan KM, Zhang Y, Long JD, Geschwind M, Kang GA, Killoran A, Lu W, McCusker E, Mills JA, Raymond LA, Testa C, Wojcieszek J, Paulsen JS, PREDICT-HD Investigators of the Huntington Study Group
Refining the diagnosis of Huntington disease: the PREDICT-HD study
Dumas EM, van den Bogaard SJ, Hart EP, Soeter RP, van Buchem MA, van der Grond J, Rombouts SA, Roos RA, TRACK-HD investigator group
Reduced functional brain connectivity prior to and after disease onset in Huntington's disease
Brooks SP, Dunnett SB
Cognitive deficits in animal models of basal ganglia disorders
Papp KV, Snyder PJ, Mills JA, Duff K, Westervelt HJ, Long JD, Lourens S, Paulsen JS
Measuring executive dysfunction longitudinally and in relation to genetic burden, brain volumetrics, and depression in prodromal Huntington disease
Georgiou-Karistianis N, Gray MA, Dominguez DJf, Dymowski AR, Bohanna I, Johnston LA, Churchyard A, Chua P, Stout JC, Egan GF
Automated differentiation of pre-diagnosis Huntington`s disease from healthy control individuals based on quadratic discriminant analysis of the basal ganglia: the IMAGE-HD study
Sathasivam K, Neueder A, Gipson TA, Landles C, Benjamin AC, Bondulich MK, Smith DL, Faull RL, Roos RA, Howland D, Detloff PJ, Housman DE, Bates GP
Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease
van den Bogaard SJ, Dumas EM, Hart EP, Milles J, Reilmann R, Stout JC, Craufurd D, Gibbard CR, Tabrizi SJ, van Buchem MA, van der Grond J, Roos RA
Magnetization transfer imaging in premanifest and manifest huntington disease: a 2-year follow-up
Southwell AL, Warby SC, Carroll JB, Doty CN, Skotte NH, Zhang W, Villanueva EB, Kovalik V, Xie Y, Pouladi MA, Collins JA, Yang XW, Franciosi S, Hayden MR
A fully humanized transgenic mouse model of Huntington disease
van Wamelen DJ, Aziz NA, Anink JJ, van Steenhoven R, Angeloni D, Fraschini F, Jockers R, Roos RA, Swaab DF
Suprachiasmatic nucleus neuropeptide expression in patients with Huntington's disease
Read J, Jones R, Owen G, Leavitt BR, Coleman A, Roos RA, Dumas EM, Durr A, Justo D, Say M, Stout JC, Tabrizi SJ, Craufurd D, Track-Hd investigators
Quality of life in Huntington`s disease: a comparative study investigating the impact for those with pre-manifest and early manifest disease, and their partners
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
Morton AJ, Howland DS
Large genetic animal models of Huntington`s Disease
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
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
2 (4) :459-75
PMID: 24696705
PMCID: PMC3970728
DOI: 10.3233/JHD-130080
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
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
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
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
Sawiak SJ, Wood NI, Carpenter TA, Morton AJ
Huntington's disease mouse models online: high-resolution MRI images with stereotaxic templates for computational neuroanatomy
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
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
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
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
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
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
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
122 (10) :3731-6
PMID: 22996692
PMCID: PMC3461928
DOI: 10.1172/JCI64565
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
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
3 (9) :731-5
PMID: 24900540
PMCID: PMC4025765
DOI: 10.1021/ml3001352
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
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
Scahill RI, Wild EJ, Tabrizi SJ
Biomarkers for Huntington`s disease: an update
Expert Opin Med Diagn
September 1, 2012
6 (5) :371-5
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
Caron NS, Munsie LN, Keillor JW, Truant R
Using FLIM-FRET to measure conformational changes of transglutaminase type 2 in live cells
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
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
HD iPSC Consortium
Induced pluripotent stem cells from patients with Huntington`s disease show CAG-repeat-expansion-associated phenotypes
Vlamings R, Zeef DH, Janssen ML, Oosterloo M, Schaper F, Jahanshahi A, Temel Y
Lessons learned from the transgenic Huntington's disease rats
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