CHDI
Listed here are over 1,100 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.
Publication Search
Title
Journal
Lee JM, Chao MJ, Harold D, Abu Elneel K, Gillis T, Holmans P, Jones L, Orth M, Myers RH, Kwak S, Wheeler VC, MacDonald ME, Gusella JF
A modifier of Huntington's disease onset at the MLH1 locus
Bresciani A, Missineo A, Gallo M, Cerretani M, Fezzardi P, Tomei L, Cicero DO, Altamura S, Santoprete A, Ingenito R, Bianchi E, Pacifici R, Dominguez C, Munoz-Sanjuan I, Harper S, Toledo-Sherman L, Park LC
Nuclear factor (erythroid-derived 2)-like 2 (NRF2) drug discovery: Biochemical toolbox to develop NRF2 activators by reversible binding of Kelch-like ECH-associated protein 1 (KEAP1)
Frost C, Mulick A, Scahill RI, Owen G, Aylward E, Leavitt BR, Durr A, Roos RAC, Borowsky B, Stout JC, Reilmann R, Langbehn DR, Tabrizi SJ, Sampaio C, TRACK-HD Investigators
Design optimization for clinical trials in early-stage manifest Huntington's disease
Niccolini F, Pagano G, Fusar-Poli P, Wood A, Mrzljak L, Sampaio C, Politis M
Striatal molecular alterations in HD gene carriers: a systematic review and meta-analysis of PET studies
Hensman Moss DJ, Pardiñas AF, Langbehn D, Lo K, Leavitt BR, Roos R, Durr A, Mead S, TRACK-HD investigators, REGISTRY investigators, Holmans P, Jones L, Tabrizi SJ
Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study
Brady ST, Morfini GA
Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases
Westervelt HJ, Bernier RA, Faust M, Gover M, Bockholt HJ, Zschiegner R, Long JD, Paulsen JS, PREDICT-HD Investigators, Coordinators of the Huntington Study Group
Data quality assurance and control in cognitive research: Lessons learned from the PREDICT-HD study
Schain M, Fazio P, Mrzljak L, Amini N, Al-Tawil N, Fitzer-Attas C, Bronzova J, Landwehrmeyer B, Sampaio C, Halldin C, Varrone A
Revisiting the Logan plot to account for non-negligible blood volume in brain tissue
Ratovitski T, O’Meally RN, Jiang M, Chaerkady R, Chighladze E, Stewart JC, Wang X, Arbez N, Roby E, Alexandris A, Duan W, Vijayvargia R, Seong IS, Lavery DJ, Cole RN, Ross CA
Post-Translational Modifications (PTMs), identified on endogenous Huntingtin, cluster within proteolytic domains between HEAT repeats
Zheng P, Kozloski J
Striatal network models of Huntington's disease dysfunction phenotypes
Ghosh S, Sun Z, Li Y, Cheng Y, Mohan A, Sampaio C, Hu J
An exploration of latent structure in observational Huntington's disease studies
Dietrich P, Johnson IM, Alli S, Dragatsis I
Elimination of huntingtin in the adult mouse leads to progressive behavioral deficits, bilateral thalamic calcification, and altered brain iron homeostasis
Kalathur RKR, Pedro Pinto J, Sahoo B, Chaurasia G, Futschik ME
HDNetDB: A molecular interaction database for network-oriented investigations into Huntington's disease
Daldin M, Fodale V, Cariulo C, Azzollini L, Verani M, Martufi P, Spiezia MC, Deguire SM, Cherubini M, Macdonald D, Weiss A, Bresciani A, Vonsattel JG, Petricca L, Marsh JL, Gines S, Santimone I, Marano M, Lashuel HA. Squitieri F, Caricasole A
Polyglutamine expansion affects huntingtin conformation in multiple Huntington's disease models
Kang H, Vázquez FX, Zhang L, Das P, Toledo-Sherman L, Luan B, Levitt M, Zhou R
Emerging β-sheet rich conformations in supercompact Huntingtin Exon-1 mutant structures
Khakh BS, Beaumont V, Cachope R, Munoz-Sanjuan I, Goldman SA, Grantyn R
Unravelling and exploiting astrocyte dysfunction in Huntington's disease
Dominguez D JF, Poudel G, Stout JC, Gray M, Chua P, Borowsky B, Egan GF, Georgiou-Karistianis N
Longitudinal changes in the fronto-striatal network are associated with executive dysfunction and behavioral dysregulation in Huntington's disease : 30 months IMAGE-HD data
Nikan M, Osborn MF, Coles AH, Biscans A, Godinho BMDC, Haraszti RA, Sapp E, Echeverria D, DiFiglia M, Aronin N, Khvorova A
Synthesis and evaluation of parenchymal retention and efficacy of a metabolically stable O-Phosphocholine-N-docosahexaenoyl-l-serine siRNA conjugate in mouse brain
Teka WW, Hamade KC, Barnett WH, Kim T, Markin SN, Rybak IA, Molkov YI
From the motor cortex to the movement and back again
Byrne LM, Rodrigues FB, Blennow K, Durr A, Leavitt BR, Roos RAC, Scahill RI, Tabrizi SJ, Zetterberg H, Langbehn D, Wild EJ
Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis
Lancet Neurol
June 7, 2017
17 (30124-2) :S1474-4422
17 (30124-2) :S1474-4422
PMID: 28601473
Quinti L, Dayalan Naidu S, Träger U, Chen X, Kegel-Gleason K Llères D, Connolly C, Chopra V, Low C, Moniot S, Sapp E, Tousley AR, Vodicka P, Van Kanegan MJ, Kaltenbach LS, Crawford LA, Fuszard M, Higgins M, Miller JRC, Farmer RE, Potluri V, Samajdar S, Meisel L, Zhang N, Snyder A, Stein R, Hersch SM, Ellerby LM, Weerapana E, Schwarzschild MA, Steegborn C, Leavitt BR, Degterev A, Tabrizi SJ, Lo DC, DiFiglia M, Thompson LM, Dinkova-Kostova AT, Kazantsev AG
KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patients
Proc Natl Acad Sci USA
June 6, 2017
114 (23) :E4676-E4685
114 (23) :E4676-E4685
PMID: 28533375
Park KHJ, Franciosi S, Parrant K, Lu G, Leavitt BR
p35 hemizygosity activates Akt but does not improve motor function in the YAC128 mouse model of Huntington's disease
Farina F, Lambert E, Commeau L, Lejeune FX, Roudier N, Fonte C, Parker JA, Boddaert J, Verny M, Baulieu EE, Neri C
The stress response factor daf-16 FOXO is required for multiple compound families to prolong the function of neurons with Huntington's disease
Cleary JD, Ranum LP
New developments in RAN translation: insights from multiple diseases
McColgan P, Razi A, Gregory S, Seunarine KK, Durr A, Roos ACR, Leavitt BR, Scahill RI, Clark CA, Langbehn DR, Rees G, Tabrizi SJ, Track On-HD Investigators
Structural and functional brain network correlates of depressive symptoms in premanifest Huntington's disease
Hum Brain Mapp
June 1, 2017
38 (6) :2819-2829
38 (6) :2819-2829
PMID: 28294457
PMCID: PMC5434856
DOI: 10.1002/hbm.23527
Fazio P, Schain M, Mrzljak L, Amini N, Nag S, Al-Tawil N, Fitzer-Attas CJ, Bronzova J, Landwehrmeyer B, Sampaio C, Halldin C, Varrone A
Patterns of age related changes for phosphodiesterase type-10A in comparison with dopamine D(2/3) receptors and sub-cortical volumes in the human basal ganglia: A PET study with (18)F-MNI-659 and (11)C-raclopride with correction for partial volume effect
Neueder A, Landles C, Ghosh R, Howland D, Myers RH, Faull RLM, Tabrizi SJ, Bates GP
The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients
Chiki A, DeGuire SM, Ruggeri FS, Sanfelice D, Ansaloni A, Wang ZM, Cendrowska U, Burai R, Vieweg S, Pastore A, Dietler G, Lashuel HA
Mutant exon1 Huntingtin aggregation is regulated by T3 phosphorylation-induced structural changes and crosstalk between T3 phosphorylation and acetylation at K6
HD iPSC Consortium
Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice
Martín-Ibáñez R, Guardia I, Pardo M, Herranz C, Zietlow R, Vinh NN, Rosser A, Canals JM
Insights in spatio-temporal characterization of human fetal neural stem cells
Coffey SR, Bragg RM, Minnig S, Ament SA, Cantle JP, Glickenhaus A, Shelnut D, Carrillo JM, Shuttleworth DD, Rodier JA, Noguchi K, Bennett CF, Price ND, Kordasiewicz HB, Carroll JB
Peripheral huntingtin silencing does not ameliorate central signs of disease in the B6.HttQ111/+ mouse model of Huntington's disease
Miller JRC, Pfister EL, Liu W, Andre R, Träger U, Kennington LA, Lo K, Dijkstra S, Macdonald D, Ostroff G, Aronin N, Tabrizi SJ
Allele-selective suppression of mutant Huntingtin in primary human blood cells
Zhang L, Kang H, Vázquez FX, Toledo-Sherman L, Luan B. Zhou R
Molecular mechanism of stabilizing the helical structure of Huntingtin N17 in a micellar environment
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