CHDI
Listed here are over 1,075 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
Murthy V, Tebaldi T, Yoshida T, Erdin S, Calzonetti T, Vijayvargia R, Tripathi T, Kerschbamer E, Seong IS, Quattrone A, Talkowski ME, Gusella JF, Georgopoulos K, MacDonald ME, Biagioli M
Hypomorphic mutation of the mouse Huntington's disease gene orthologue
Merienne N, Meunier C, Schneider A, Seguin J, Nair SS, Rocher AB, Le Gras S, Keime C, Faull R, Pellerin L, Chatton JY, Neri C, Merienne K, Déglon N
Cell-type-specific gene expression profiling in adult mouse brain reveals normal and disease-state signatures
Kang R, Wang L, Sanders SS, Zuo K, Hayden MR, Raymond LA
Altered regulation of striatal neuronal N-Methyl-D-Aspartate receptor trafficking by palmitoylation in Huntington disease mouse model
Castaldo I, De Rosa M, Romano A, Zuchegna C, Squitieri F, Mechelli R, Peluso S, Borrelli C, Del Mondo A, Salvatore E, Vescovi LA, Migliore S, De Michele G, Ristori G, Romano S, Avvedimento EV, Porcellini A
DNA damage signatures in peripheral blood cells as biomarkers in prodromal huntington disease
Tousley A, Iuliano M, Weisman E, Sapp E, Richardson H, Vodicka P, Alexander J, Aronin N, DiFiglia M, Kegel-Gleason KB
Huntingtin associates with the actin cytoskeleton and α-actinin isoforms to influence stimulus dependent morphology changes
Goold R, Flower M, Moss DH, Medway C, Wood-Kaczmar A, Andre R, Farshim P, Bates GP, Holmans P, Jones L, Tabrizi SJ
FAN1 modifies Huntington's disease progression by stabilizing the expanded HTT CAG repeat
Hum Mol Genet
February 15, 2019
28 (4) :650-661
28 (4) :650-661
PMID: 30358836
PMCID: PMC6360275
DOI: 10.1093/hmg/ddy375
Tousley A, Iuliano M, Weisman E, Sapp E, Zhang N, Vodicka P, Alexander J, Aviolat H, Gatune L, Reeves P, Li X, Khvorova A, Ellerby LM, Aronin N, DiFiglia M, Kegel-Gleason KB
Rac1 activity is modulated by huntingtin and dysregulated in models of Huntington's disease
J Huntingtons Dis
February 13, 2019
8 (1) :53-69
8 (1) :53-69
PMID: 30594931
PMCID: 6398565
DOI: 10.3233/JHD-180311
Rieke L, Fels M, Schubert R, Habbel B, Matheis T, Schuldenzucker V, Kemper N, Reilmann R
Activity behaviour of minipigs transgenic for the Huntington gene
Smatlikova P, Juhas S, Juhasova J, Suchy T, Hubalek Kalbacova M, Ellederova Z, Motlik J, Klima J
Adipogenic differentiation of bone marrow-derived mesenchymal stem cells in pig transgenic model expressing human mutant huntingtin
Perez-Rosello T, Gelman S, Tombaugh G, Cachope R, Beaumont V, Surmeier DJ
Enhanced striatopallidal gamma-aminobutyric acid (GABA)(A) receptor transmission in mouse models of huntington's disease
Ciarochi JA, Johnson HJ, Calhoun VD, Liu J, Espinoza FA, Bockholt HJ, Misiura M, Caprihan A, Plis S, Paulsen JS, Turner JA, PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Concurrent cross-sectional and longitudinal analyses of multivariate white matter profiles and clinical functioning in pre-diagnosis Huntington disease
Paulsen JS, Lourens S, Kieburtz K, Zhang Y
Sample enrichment for clinical trials to show delay of onset in Huntington disease
Padovan-Neto FE, Jurkowski L, Murray C, Stutzmann GE, Kwan M, Ghavami A, Beaumont V, Park LC, West AR
Age- and sex-related changes in cortical and striatal nitric oxide synthase in the Q175 mouse model of Huntington's disease
Holley SM, Galvan L, Kamdjou T, Cepeda C, Levine MS
Striatal GABAergic interneuron dysfunction in the Q175 mouse model of Huntington's disease
Rodrigues FB, Byrne LM, De Vita E, Johnson EB, Hobbs NZ, Thornton JS, Scahill RI, Wild EJ
Cerebrospinal fluid flow dynamics in Huntington's disease evaluated by phase contrast MRI
Sun Z, Ghosh S, Li Y, Cheng Y, Mohan A, Sampaio C, Hu J
A probabilistic disease progression modeling approach and its application to integrated Huntington's disease observational data
Lu M, Banetta L, Young LJ, Smith EJ, Bates GP, Zaccone A, Kaminski Schierle GS, Tunnacliffe A, Kaminski CF
Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assembly
Osipovitch M, Asenjo Martinez A, Mariani JN, Cornwell A, Dhaliwal S, Zou L, Chandler-Militello D, Wang S, Li X, Benraiss SJ, Agate R, Lampp A, Benraiss A, Windrem MS, Goldman SA
Human ESC-derived chimeric mouse models of Huntington's disease reveal cell-intrinsic defects in glial progenitor cell differentiation
Luckhurst CA, Aziz O, Beaumont V, Bürli RW, Breccia P, Maillard MC, Haughan AF, Lamers M, Leonard P, Matthews KL, Raphy G, Stott AJ, Munoz-Sanjuan I, Thomas B, Wall M, Wishart G, Yates D, Dominguez C
Development and characterization of a CNS-penetrant benzhydryl hydroxamic acid class IIa histone deacetylase inhibitor
Naze S, Humble J, Zheng P, Barton S, Rangel-Barajas C, Rebec GV, Kozloski JR
Cortico-striatal cross-frequency coupling and gamma genesis disruptions in Huntington's disease mouse and computational models
Bravo-Arredondo JM, Kegulian NC, Schmidt T, Pandey NK, Situ AJ, Ulmer TS, Langen R
The folding equilibrium of huntingtin exon 1 monomer depends on its polyglutamine tract
Wang D, Zhong L, Li M, Li J, Tran K, Ren L, He R, Xie J, Moser RP, Fraser C, Kuchel T, Sena-Esteves M, Flotte TR, Aronin N, Gao G
Adeno-associated virus neutralizing antibodies in large animals and their impact on brain intraparenchymal gene transfer
McBride SD, Morton AJ
Indices of comparative cognition: assessing animal models of human brain function
Kuljis D, Kudo T, Tahara Y, Ghiani CA, Colwell CS
Pathophysiology in the suprachiasmatic nucleus in mouse models of Huntington's disease
Mondo E, Moser R, Gao G, Mueller C, Sena-Esteves M, Sapp E, Pfister E, O’Connell D, Takle K, Erger KE, Liu W, Conlon TJ, DiFiglia M, Gounis MJ, Aronin N
Selective neuronal uptake and distribution of AAVrh8, AAV9, and AAVrh10 in sheep after intra-striatal administration
Garcia TP, Wang Y, Shoulson I, Paulsen JS, Marder K
Disease progression in Huntington disease: an analysis of multiple longitudinal outcomes
Long JD, Mills JA
Joint modeling of multivariate longitudinal data and survival data in several observational studies of Huntington's disease
Godinho BMDC, Henninger N, Bouley J, Alterman JF, Haraszti RA, Gilbert JW, Sapp E, Coles AH, Biscans A, Nikan M, Echeverria D, DiFiglia M, Aronin N, Khvorova A
Transvascular delivery of hydrophobically modified siRNAs: gene silencing in the rat brain upon disruption of the blood-brain barrier
Rocha NP, Mwangi B, Gutierrez Candano CA, Sampaio C, Furr Stimming E, Teixeira AL
The clinical picture of psychosis in manifest Huntington's disease: a comprehensive analysis of the Enroll-HD database
Rowley CD, Tabrizi SJ, Scahill RI, Leavitt BR, Roos RAC, Durr A, Bock NA
Altered intracortical T(1)-weighted/T(2)-weighted ratio signal in Huntington's disease
Zhang J, Gregory S, Scahill RI, Durr A, Thomas DL, Lehericy S, Rees G, Tabrizi SJ, Zhang H, TrackOn-HD investigators
In vivo characterization of white matter pathology in premanifest huntington's disease
Bertoglio D, Verhaeghe J, Kosten L, Thomae D, Van der Linden A, Stroobants S, Wityak J, Dominguez C, Mrzljak L, Staelens S
MR-based spatial normalization improves [18F]MNI-659 PET regional quantification and detectability of disease effect in the Q175 mouse model of Huntington's disease
Piiponniemi TO, Parkkari T, Heikkinen T, Puoliväli J, Park LC, Cachope R, Kopanitsa MV
Impaired performance of the Q175 mouse model of Huntington's disease in the touch screen paired associates learning task
Aron R, Pellegrini P, Green EW, Maddison DC, Opoku-Nsiah K, Wong JS, Daub AC, Giorgini F, Finkbeiner S
Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's disease
Neueder A, Dumas AA, Benjamin AC, Bates GP
Regulatory mechanisms of incomplete huntingtin mRNA splicing
Byrne LM, Rodrigues FB, Johnson EB, Wijeratne PA, De Vita E, Alexander DC, Palermo G, Czech C, Schobel S, Scahill RI, Heslegrave A, Zetterberg H, Wild EJ
Evaluation of mutant huntingtin and neurofilament proteins as potential markers in Huntington's disease
Sci Transl Med
September 12, 2018
10 (458) :eaat7108
10 (458) :eaat7108
PMID: 30209243
Siebzehnrübl FA, Raber KA, Urbach YK, Schulze-Krebs A, Canneva F, Moceri S, Habermeyer J, Achoui D, Gupta B, Steindler DA, Stephan M, Nguyen HP, Bonin M, Riess O, Bauer A, Aigner L, Couillard-Despres S, Paucar MA, Svenningsson P, Osmand A, Andreew A, Zabel C, Weiss A, Kuhn R, Moussaoui S, Blockx I, Van der Linden A, Cheong RY, Roybon L, Petersén Å, von Hörsten S
Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition
Ast A, Buntru A, Schindler F, Hasenkopf R, Schulz A, Brusendorf L, Klockmeier K, Grelle G, McMahon B, Niederlechner H, Jansen I, Diez L, Edel J, Boeddrich A, Franklin SA, Baldo B, Schnoegl S, Kunz S, Purfürst B, Gaertner A, Kampinga HH, Morton AJ, Petersén Å, Kirstein J, Bates GP, Wanker EE
mHTT seeding activity: a marker of disease progression and neurotoxicity in models of Huntington's disease
Evers MM, Miniarikova J, Juhas S, Vallès A, Bohuslavova B, Juhasova J, Skalnikova HK, Vodicka P, Valekova I, Brouwers C, Blits B, Lubelski J, Kovarova H, Ellederova Z, van Deventer SJ, Petry H, Motlik J, Konstantinova P
AAV5-miHTT gene therapy demonstrates broad distribution and strong human mutant huntingtin lowering in a Huntington's disease minipig model
Didiot MC, Ferguson CM, Ly S, Coles AH, Smith AO, Bicknell AA, Hall LM, Sapp E, Echeverria D, Pai AA, DiFiglia M, Moore MJ, Hayward LJ, Aronin N, Khvorova A
Nuclear localization of Huntingtin mRNA is specific to cells of neuronal origin