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
Gasset-Rosa F, Chillon-Marinas C, Goginashvili A, Atwal RS, Artates JW, Tabet R, Wheeler VC, Bang AG, Cleveland DW, Lagier-Tourenne C
Polyglutamine-expanded huntingtin exacerbates age-related disruption of nuclear integrity and nucleocytoplasmic transport
Gregory S, Long JD, Klöppel S, Razi A, Scheller E, Minkova L, Papoutsi M, Mills JA, Durr A, Leavitt BR, Roos RAC, Stout JC, Scahill RI, Langbehn DR, Tabrizi SJ, Rees G
Operationalizing compensation over time in neurodegenerative disease
Häggkvist J, Tóth M, Tari L, Varnäs K, Svedberg M, Forsberg A, Nag S, Dominguez C, Munoz-Sanjuan I, Bard J, Wityak J, Varrone A, Halldin C, Mrzljak L
Longitudinal small-animal PET imaging of the zQ175 mouse model of Huntington disease shows in vivo changes of molecular targets in the striatum and cerebral cortex
Wang JKT, Langfelder P, Horvath S, Palazzolo MJ
Exosomes and homeostatic synaptic plasticity are linked to each other and to Huntington's, Parkinson's, and other neurodegenerative diseases by database-enabled analyses of comprehensively curated datasets
Kim T, Hamade KC, Todorov D, Barnett WH, Capps RA, Latash EM, Markin SN, Rybak IA, Molkov YI
Reward based motor adaptation mediated by basal ganglia
Liu JP, Zeitlin SO
Is Huntingtin dispensable in the adult brain?
J Huntingtons Dis
March 27, 2017
6 (1) :43847
6 (1) :43847
PMID: 28339401
PMCID: PMC5389021
DOI: 10.3233/JHD-170235
Hensman Moss DJ, Flower MD, Lo KK, Miller JR, van Ommen GB, ‘t Hoen PA, Stone TC, Guinee A, Langbehn DR, Jones L, Plagnol V, van Roon-Mom WM, Holmans P, Tabrizi SJ
Huntington's disease blood and brain show a common gene expression pattern and share an immune signature with Alzheimer's disease
Southwell AL, Skotte NH, Villanueva EB, Østergaard ME, Gu X, Kordasiewicz HB, Kay C, Cheung D, Xie Y, Waltl S, Dal Cengio L, Findlay-Black H, Doty CN, Petoukhov E, Iworima D, Slama R, Ooi J, Pouladi MA, Yang XW, Swayze EE, Seth PP, Hayden MR
A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles
Ament SA, Pearl JR, Grindeland A, St Claire J, Earls JC, Kovalenko M, Gillis T, Mysore J, Gusella JF, Lee JM, Kwak S, Howland D, Lee MY, Baxter D, Scherler K, Wang K, Geman D, Carroll JB, MacDonald ME, Carlson G, Wheeler VC, Price ND, Hood LE
High resolution time-course mapping of early transcriptomic, molecular and cellular phenotypes in Huntington's disease CAG knock-in mice across multiple genetic backgrounds
Schultz JL, Kamholz JA, Moser DJ, Feely SM, Paulsen JS, Nopoulos PC
Substance abuse may hasten motor onset of Huntington disease: Evaluating the Enroll-HD database
Yu MS, Tanese N
Huntingtin is required for neural but not cardiac/pancreatic progenitor differentiation of mouse embryonic stem cells in vitro
Long JD, Langbehn DR, Tabrizi SJ, Landwehrmeyer BG, Paulsen JS, Warner J, Sampaio C
Validation of a prognostic index for Huntington's disease
Mov Disord
February 20, 2017
32 (2) :256-263
32 (2) :256-263
PMID: 27892614
PMCID: PMC5318276
DOI: 10.1002/mds.26838
Skene DJ, Middleton B, Fraser CK, Pennings JL, Kuchel TR, Rudiger SR, Bawden CS, Morton AJ
Metabolic profiling of presymptomatic Huntington's disease sheep reveals novel biomarkers
Sci Reports
February 17, 2017
7 (43030) :43846
7 (43030) :43846
PMID: 28223686
PMCID: PMC5320451
DOI: 10.1038/srep43030
Faedo A, Laporta A, Segnali A, Galimberti M, Besusso D, Cesana E, Belloli S, Moresco RM, Tropiano M, Fucà E, Wild S, Bosio A, Vercelli AE, Biella G, Cattaneo E
Differentiation of human telencephalic progenitor cells into MSNs by inducible expression of Gsx2 and Ebf1
Shin A, Shin B, Shin JW, Kim KH, Atwal RS, Hope JM, Gillis T, Leszyk JD, Shaffer SA, Lee R, Kwak S, MacDonald ME, Gusella JF, Seong IS, Lee JM
Novel allele-specific quantification methods reveal no effects of adult onset CAG repeats on HTT mRNA and protein levels
Shankaran M, Di Paolo E, Leoni V, Caccia C, Ferrari Bardile C, Mohammed H, Di Donato S, Kwak S, Marchionini D, Turner S, Cattaneo E, Valenza M
Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice
McColgan P, Gregory S, Razi A, Seunarine KK, Gargouri F, Durr A, Roos RA, Leavitt BR, Scahill RI, Clark CA, Tabrizi SJ, Rees G, Track On‐HD Investigators, Coleman A, Decolongon J, Fan M, Petkau T, Jauffret C, Justo D, Lehericy S, Nigaud K, Valabrègue R, Choonderbeek A, Hart EP, Hensman Moss DJ, Crawford H, Johnson E, Papoutsi M, Berna C, Reilmann R, Weber N, Stout J, Labuschagne I, Landwehrmeyer B, Orth M, Johnson H
White matter predicts functional connectivity in premanifest Huntington's disease
Ann Clin Transl Neurol
January 16, 2017
4 (2) :106-118
4 (2) :106-118
PMID: 28168210
PMCID: 5288460
DOI: 10.1002/acn3.384
Maiuri T, Mocle AJ, Hung CL, Xia J, van Roon-Mom WM, Truant R
Huntingtin is a scaffolding protein in the ATM oxidative DNA damage response complex
Lee JK, Ding Y, Conrad AL, Cattaneo E, Epping E, Mathews K, Gonzalez-Alegre P, Cahill L, Magnotta V, Schlaggar BL, Perlmutter JS, Kim RE, Dawson JD, Nopoulos P
Sex-specific effects of the Huntington gene on normal neurodevelopment
Orth M, Gregory S, Scahill RI, Mayer IS, Minkova L, Klöppel S, Seunarine KK, Boyd L, Borowsky B, Reilmann R, Landwehrmeyer BG, Leavitt BR, Roos RA, Durr A, Rees G, Rothwell JC, Langbehn D, Tabrizi SJ, TRACK-On Investigators
Natural variation in sensory-motor white matter organization influences manifestations of Huntington's disease
Beaumont V, Zhong S, Lin H, Xu W, Bradaia A, Steidl E, Gleyzes M, Wadel K, Buisson B, Padovan-Neto FE, Chakroborty S, Ward KM, Harms JF, Beltran J, Kwan M, Ghavami A, Häggkvist J, Tóth M, Halldin C, Varrone A, Schaab C, Dybowski JN, Elschenbroich S, Lehtimäki K, Heikkinen T, Park L, Rosinski J, Mrzljak L, Lavery D, West AR, Schmidt CJ, Zaleska MM, Munoz-Sanjuan I
Phosphodiesterase 10A inhibition improves cortico-basal ganglia function in Huntington's disease models
Atherton JF, McIver EL, Mullen MR, Wokosin DL, Surmeier DJ, Bevan MD
Early dysfunction and progressive degeneration of the subthalamic nucleus in mouse models of Huntington's disease
Rangel-Barajas C, Rebec GV
Dysregulation of corticostriatal connectivity in Huntington's disease: A role for dopamine modulation
J Huntingtons Dis
December 15, 2016
5 (4) :303-331
5 (4) :303-331
PMID: 27983564
PMCID: 5181679
DOI: 10.3233/JHD-160221
Deng YP, Reiner A
Cholinergic interneurons in the Q140 knockin mouse model of Huntington's disease: Reductions in dendritic branching and thalamostriatal input
J Comp Neurol
December 1, 2016
524 (17) :3518-3529
524 (17) :3518-3529
PMID: 27219491
PMCID: PMC5050058
DOI: 10.1002/cne.24013
Didiot MC, Hall LM, Coles AH, Haraszti RA, Godinho BM, Chase K, Sapp E, Ly S, Alterman JF, Hassler MR, Echeverria D, Raj L, Morrissey DV, DiFiglia M, Aronin N, Khvorova A
Exosome-mediated delivery of hydrophobically modified siRNA for huntingtin mRNA silencing
Sawiak SJ, Wood NI, Morton AJ
Similar progression of morphological and metabolic phenotype in R6/2 mice with different CAG repeats revealed by in vivo magnetic resonance imaging and spectroscopy
Keeler AM, Sapp E, Chase K, Sottosanti E, Danielson E, Pfister E, Stoica L, DiFiglia M, Aronin N, Sena-Esteves M
Cellular analysis of silencing the Huntington's disease gene using AAV9 mediated delivery of artificial micro RNA into the Striatum of Q140/Q140 mice
Ciarochi JA, Calhoun VD, Lourens S, Long JD, Johnson HJ, Bockholt HJ, Liu J, Plis SM, Paulsen JS, Turner JA, PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Patterns of co-occurring gray matter concentration loss across the Huntington disease prodrome
Verani M, Bustamante M, Martufi P, Daldin M, Cariulo C, Azzollini L, Fodale V, Puglisi F, Weiss A, Macdonald D, Petricca L, Caricasole A
Conformational modulation mediated by polyglutamine expansion in CAG repeat expansion disease-associated proteins
Biochem Biophys Res Commun
September 16, 2016
478 (2) :949-55
478 (2) :949-55
PMID: 27520369
Naseri NN, Bonica J, Xu H, Park LC, Arjomand J, Chen Z, Gibson GE
Novel metabolic abnormalities in the tricarboxylic acid cycle in peripheral cells from Huntington's disease patients
Ruggeri FS, Vieweg S, Cendrowska U, Longo G, Chiki A, Lashuel HA, Dietler G
Nanoscale studies link amyloid maturity with polyglutamine diseases onset
Warner JH, Sampaio C
Modeling variability in the progression of Huntington`s disease a novel modeling approach applied to structural imaging markers from TRACK-HD
CPT Pharmacometrics Syst Pharmacol
August 2, 2016
5 (8) :437-45
5 (8) :437-45
PMID: 27481337
PMCID: PMC4999607
DOI: 10.1002/psp4.12097
Beaumont V, Mrzljak L, Dijkman U, Freije R, Heins M, Rassoulpour A, Tombaugh G, Gelman S, Bradaia A, Steidl E, Gleyzes M, Heikkinen T, Lehtimaki K, Puolivali J, Kontkanen O, Javier RM, Neagoe I, Deisemann H, Winkler D, Ebneth A, Khetarpal V, Toledo-Sherman L, Dominguez C, Park LC, Munoz-Sanjuan I
The novel KMO inhibitor CHDI-340246 leads to a restoration of electrophysiological alterations in mouse models of Huntington`s disease
Turner LM, Jakabek D, Wilkes FA, Croft RJ, Churchyard A, Walterfang M, Velakoulis D, Looi JC, Georgiou-Karistianis N, Apthorp D
Striatal morphology correlates with frontostriatal electrophysiological motor processing in Huntington's disease: an IMAGE-HD study
Cockerham R, Liu S, Cachope R, Kiyokage E, Cheer JF, Shipley MT, Puche AC
Subsecond regulation of synaptically released dopamine by COMT in the olfactory bulb
Alexandrov V, Brunner D, Menalled LB, Kudwa A, Watson-Johnson J, Mazzella M, Russell I, Ruiz MC, Torello J, Sabath E, Sanchez A, Gomez M, Filipov I, Cox K, Kwan M, Ghavami A, Ramboz S, Lager B, Wheeler VC, Aaronson J, Rosinski J, Gusella JF, MacDonald ME, Howland D, Kwak S
Large-scale phenome analysis defines a behavioral signature for Huntington`s disease genotype in mice
Horvath S, Langfelder P, Kwak S, Aaronson J, Rosinski J, Vogt TF, Eszes M, Faull RL, Curtis MA, Waldvogel HJ, Choi OW, Tung S, Vinters HV, Coppola G, Yang XW
Huntington`s disease accelerates epigenetic aging of human brain and disrupts DNA methylation levels
Labuschagne I, Cassidy AM, Scahill RI, Johnson EB, Rees E, O’Regan A, Queller S, Frost C, Leavitt BR, Dürr A, Roos R, Owen G, Borowsky B, Tabrizi SJ, Stout JC, TRACK-HD Investigators
Visuospatial processing deficits linked to posterior brain regions in premanifest and early stage Huntington's disease
Frich JC, Rae D, Roxburgh R, Miedzybrodzka ZH, Edmondson M, Pope EB, Goodman L, Haddad MS, Giuliano J, Nelson EC, Guttman M, Nance M
Health care delivery practices in Huntington`s disease specialty clinics: an international survey
Landwehrmeyer GB, Fitzer-Attas CJ, Giuliano JD, Gonçalves N, Anderson KE, Cardoso F, Ferreira JJ, Mestre TA, Stout JC, Sampaio C
Data analytics from Enroll-HD, a global clinical research platform for Huntington's disease
Mov Disord Clin Pract
June 22, 2016
4 (2) :212-224
4 (2) :212-224
PMID: 30363395
PMCID: PMC6174428
DOI: 10.1002/mdc3.12388