CHDI
Listed here are over 1000 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
Huhtala T, Poutiainen P, Rytkönen J, Lehtimäki K, Parkkari T, Kasanen I, Airaksinen AJ, Koivula T, Sweeney P, Kontkanen O, Wityak J, Dominiquez C, Park LC
Improved synthesis of [(18)F] fallypride and characterization of a Huntington's disease mouse model, zQ175DN KI, using longitudinal PET imaging of D2/D3 receptors
Alterman JF, Godinho BMDC, Hassler MR, Ferguson CM, Echeverria D, Sapp E, Haraszti RA, Coles AH, Conroy F, Miller R, Roux L, Yan P, Knox EG, Turanov AA, King RM, Gernoux G, Mueller C, Gray-Edwards HL, Moser RP, Bishop NC, Jaber SM, Gounis MJ, Sena-Esteves M, Pai AA, DiFiglia M, Aronin N, Khvorova A
A divalent siRNA chemical scaffold for potent and sustained modulation of gene expression throughout the central nervous system
Gatto RG, Ye AQ, Colon-Perez L, Mareci TH, Lysakowski A, Price SD, Brady ST, Karaman M, Morfini G, Magin RL
Detection of axonal degeneration in a mouse model of Huntington's disease: comparison between diffusion tensor imaging and anomalous diffusion metrics
Kacher R, Lamazière A, Heck N, Kappes V, Mounier C, Despres G, Dembitskaya Y, Perrin E, Christaller W, Sasidharan Nair S, Messent V, Cartier N, Vanhoutte P, Venance L, Saudou F, Néri C, Caboche J, Betuing S
CYP46A1 gene therapy deciphers the role of brain cholesterol metabolism in Huntington's disease
Bertoglio D, Verhaeghe J, Miranda A, Kertesz I, Cybulska K, Korat Š, Wyffels L, Stroobants S, Mrzljak L, Dominguez C, Liu L, Skinbjerg M, Munoz-Sanjuan I, Staelens S
Validation and noninvasive kinetic modeling of [(11) C] UCB-J PET imaging in mice
Flower M, Lomeikaite V, Ciosi M, Cumming S, Morales F, Lo K, Hensman Moss D, Jones L, Holmans P, TRACK-HD Investigators, OPTIMISTIC Consortium, Monckton DG, Tabrizi SJ
MSH3 modifies somatic instability and disease severity in Huntington's and myotonic dystrophy type 1
Wilkes FA, Abaryan Z, Ching CRK, Gutman BA, Madsen SK, Walterfang M, Velakoulis D, Stout JC, Chua P, Egan GF, Thompson PM, Looi JCL, Georgiou-Karistianis N
Striatal morphology and neurocognitive dysfunction in Huntington disease: the IMAGE-HD study
Psychiatry Res Neuroimaging
July 12, 2019
291 :43838
291 :43838
PMID: 31330407
Franich NR, Hickey MA, Zhu C, Osborne GF, Ali N, Chu T, Bove NH, Lemesre V, Lerner RP, Zeitlin SO, Howland D, Neueder A, Landles C, Bates GP, Chesselet MF
Phenotype onset in Huntington's disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene
Shishegar R, Rajapakse S, Georgiou-Karistianis N
Altered cortical morphometry in pre-manifest Huntington's disease: Cross-sectional data from the IMAGE-HD study
Annu Int Conf IEEE Eng Med Biol
July 1, 2019
2019 :2844-2847
2019 :2844-2847
PMID: 31946485
Zeitler B, Froelich S, Marlen K, Shivak DA, Yu Q, Li D, Pearl JR, Miller JC, Zhang L, Paschon DE, Hinkley SJ, Ankoudinova I, Lam S, Guschin D, Kopan L, Cherone JM, Nguyen HB, Qiao G, Ataei Y, Mendel MC, Amora R, Surosky R, Laganiere J, Vu BJ, Narayanan A, Sedaghat Y, Tillack K, Thiede C, Gärtner A, Kwak S, Bard J, Mrzljak L, Park L, Heikkinen T, Lehtimäki KK, Svedberg MM, Häggkvist J, Tari L, Tóth M, Varrone A, Halldin C, Kudwa AE, Ramboz S, Day M, Kondapalli J, Surmeier DJ, Urnov FD, Gregory PD, Rebar EJ, Muñoz-Sanjuán I, Zhang HS
Allele-selective transcriptional repression of mutant HTT for the treatment of Huntington's disease
Park S, Colwell CS
Do disruptions in the circadian timing system contribute to autonomic dysfunction in Huntington's disease?
Evans AH, Okai D, Weintraub D, Lim SY, O’Sullivan SS, Voon V, Krack P, Sampaio C, Post B, Leentjens AFG, Martinez-Martin P, Stebbins GT, Goetz CG, Schrag A, IPMDS Rating Scales Review Committee
Scales to assess impulsive and compulsive behaviors in Parkinson's disease: Critique and recommendations
Huguet G, Temel Y, Kádár E, Pol S, Casaca-Carreira J, Segura-Torres P, Jahanshahi A
Altered expression of dopaminergic cell fate regulating genes prior to manifestation of symptoms in a transgenic rat model of Huntington's disease
Ochaba J, Fote G, Kachemov M, Thein S, Yeung SY, Lau AL, Hernandez S, Lim RG, Casale M, Neel MJ, Monuki ES, Reidling J, Housman DE, Thompson LM, Steffan JS
IKKβ slows Huntington's disease progression in R6/1 mice
Proc Natl Acad Sci USA
May 28, 2019
116 (22) :10952-10961
116 (22) :10952-10961
PMID: 31088970
Octeau JC, Gangwani MR, Allam SL, Tran D, Huang S, Hoang-Trong TM, Golshani P, Rumbell TH, Kozloski JR, Khakh BS
Transient, consequential increases in extracellular potassium ions accompany Channelrhodopsin2 excitation
Dvorzhak A, Helassa N, Török K, Schmitz D, Grantyna R
Single synapse indicators of impaired glutamate clearance derived from fast iGlu u imaging of cortical afferents in the striatum of normal and Huntington (Q175) mice
Svedberg MM, Varnäs K, Varrone A, Mitsios N, Mulder J, Gulyás B, Beaumont V, Munoz-Sanjuan I, Zaleska MM, Schmidt CJ, Halldin C, Mrzljak L
In vitro phosphodiesterase 10A (PDE10A) binding in whole hemisphere human brain using the PET radioligand [(18)F] MNI-659
Holley SM, Galvan L, Kamdjou T, Dong A, Levine MS, Cepeda C
Major contribution of somatostatin-expressing interneurons and cannabinoid receptors to increased GABA synaptic activity in the striatum of Huntington's disease mice
Pido-Lopez J, Tanudjojo B, Farag S, Bondulich MK, Andre R, Tabrizi SJ, Bates GP
Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington's disease by etanercept treatment
Sharma M, Subramaniam S
Rhes travels from cell to cell and transports Huntington disease protein via TNT-like protrusion
Shahn Z, Li Y, Sun Z, Mohan A, Sampaio C, Hu J
G-Computation and hierarchical models for estimating multiple causal effects from observational disease registries with irregular visits
Di Marco A, Gonzalez Paz O, Fini I, Vignone D, Cellucci A, Battista MR, Auciello G, Orsatti L, Zini M, Monteagudo E, Khetarpal V, Rose M, Dominguez C, Herbst T, Toledo-Sherman L, Summa V, Muñoz-Sanjuán I
Application of an in vitro blood-brain barrier model in the selection of experimental drug candidates for the treatment of Huntington's disease
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