CHDI
Listed here are over 1,045 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
Shoulson I, Eberly S, Oakes D, Kayson E, Young AB PHAROS Investigators
Phenotype-genotype discrepancies in the prospective Huntington at-risk observational study
Ann Clin Transl Neurol
May 3, 2019
6 (6) :1046-1052
6 (6) :1046-1052
PMID: 31211168
PMCID: PMC6562068
DOI: 10.1002/acn3.781
Vizcarra JA, Sánchez-Ferro Á, Maetzler W, Marsili L, Zavala L, Lang AE, Martinez-Martin P, Mestre TA, Reilmann R, Hausdorff JM, Dorsey ER, Paul SS, Dexheimer JW, Wissel BD, Fuller RLM, Bonato P, Tan AH, Bloem BR, Kopil C, Daeschler M, Bataille L, Kleiner G, Cedarbaum JM, Klucken J, Merola A, Goetz CG, Stebbins GT, Espay AJ MDS Technology Task Force and MDS Rating Scales Program Electronic Development Ad-Hoc Committee
The Parkinson's disease e-diary: Developing a clinical and research tool for the digital age
Kojer K, Hering T, Bazenet C, Weiss A, Herrmann F, Taanman JW, Orth M
Huntingtin aggregates and mitochondrial pathology in skeletal muscle but not heart of late-stage R6/2 mice
Harding RJ, Loppnau P, Ackloo S, Lemak A, Hutchinson A, Hunt B, Holehouse AS, Ho JC, Fan L, Toledo-Sherman L, Seitova A, Arrowsmith CH
Design and characterization of mutant and wildtype huntingtin proteins produced from a toolkit of scalable eukaryotic expression systems
Carrillo-Reid L, Day M, Xie Z, Melendez AE, Kondapalli J, Plotkin JL, Wokosin DL, Chen Y, Kress GJ, Kaplitt M, Ilijic E, Guzman JN, Chan SS, Surmeier DJ
Mutant huntingtin enhances activation of dendritic Kv4 K(+) channels in striatal spiny projection neurons
Espinoza FA, Liu J, Ciarochi J, Turner JA, Vergara VM, Caprihan A, Misiura M, Johnson HJ, Long JD, Bockholt JH, Paulsen JS, Calhoun VD
Dynamic functional network connectivity in Huntington's disease and its associations with motor and cognitive measures
Todorov DI, Capps RA, Barnett WH, Latash EM, Kim T, Hamade KC, Markin SN, Rybak IA, Molkov YI
The interplay between cerebellum and basal ganglia in motor adaptation: A modeling study
Tang X, Ross CA, Johnson H, Paulsen JS, Younes L, Albin RL, Ratnanather JT, Miller MI
Regional subcortical shape analysis in premanifest Huntington's disease
Toledo-Sherman L, Breccia P, Cachope R, Bate JR, Angulo-Herrera I, Wishart G, Matthews KL, Martin SL, Cox HC, McAllister G, Penrose SD, Vater H, Esmieu W, Van de Poël A, Van de Bospoort R, Strijbosch A, Lamers M, Leonard P, Jarvis RE, Blackaby W, Barnes K, Eznarriaga M, Dowler S, Smith GD, Fischer DF, Lazari O, Yates D, Rose M, Jang SW, Muñoz-Sanjuan I, Dominguez C
Optimization of potent and selective ataxia telangiectasia-mutated inhibitors suitable for a proof-of-concept study in Huntington's disease models
Reindl W, Baldo B, Schulz J, Janack I, Lindner I, Kleinschmidt M, Sedaghat Y, Thiede C, Tillack K, Schmidt C, Cardaun I, Schwagarus T, Herrmann F, Hotze M, Osborne GF, Herrmann S, Weiss A, Zerbinatti C, Bates GP, Bard J, Munoz-Sanjuan I, Macdonald D
Meso scale discovery-based assays for the detection of aggregated huntingtin
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