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
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
Goodliffe JW, Song H, Rubakovic A, Chang W, Medalla M, Weaver CM, Luebke JI
Differential changes to D1 and D2 medium spiny neurons in the 12-month-old Q175+/- mouse model of Huntington's disease
Haraszti RA, Miller R, Didiot MC, Biscans A, Alterman JF, Hassler MR, Roux L, Echeverria D, Sapp E, DiFiglia M, Aronin N, Khvorova A
Optimized cholesterol-siRNA chemistry improves productive loading onto extracellular vesicles
Rieke L, Schubert R, Matheis T, Muratori LM, Motlik J, Schramke S, Fels M, Kemper N, Schuldenzucker V, Reilmann R
Vocalisation as a viable assessment for phenotyping minipigs transgenic for the Huntington gene?
McNulty P, Pilcher R, Ramesh R, Necuiniate R, Hughes A, Farewell D, Holmans P, Jones L, REGISTRY Investigators of the European Huntington’s Disease Network
Reduced cancer incidence in Huntington's disease: Analysis in the Registry study
Wesson M, Boileau NR, Perlmutter JS, Paulsen JS, Barton SK, McCormack MK, Carlozzi NE
Suicidal ideation assessment in individuals with premanifest and manifest Huntington disease
J Huntingtons Dis
July 31, 2018
7 (3) :239-249
7 (3) :239-249
PMID: 30056431
PMCID: PMC6108173
DOI: 10.3233/JHD-180299
Pido-Lopez J, Andre R, Benjamin AC, Ali N, Farag S, Tabrizi SJ, Bates GP
In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington's disease
Mir A, Alterman JF, Hassler MR, Debacker AJ, Hudgens E, Echeverria D, Brodsky MH, Khvorova A, Watts JK, Sontheimer EJ
Heavily and fully modified RNAs guide efficient SpyCas9-mediated genome editing
Gregory S, Long JD, Klöppel S, Razi A, Scheller E, Minkova L, Johnson EB, Durr A, Roos RAC, Leavitt BR, Mills JA, Stout JC, Scahill RI, Tabrizi SJ, Rees G, Track-On investigators
Testing a longitudinal compensation model in premanifest Huntington's disease
Ko J, Isas JM, Sabbaugh A, Yoo JH, Pandey NK, Chongtham A, Ladinsky M, Wu WL, Rohweder H, Weiss A, Macdonald D, Munoz-Sanjuan I, Langen R, Patterson PH, Khoshnan A
Identification of distinct conformations associated with monomers and fibril assemblies of mutant huntingtin
Fritz NE, Boileau NR, Stout JC, Ready R, Perlmutter JS, Paulsen JS, Quaid K, Barton S, McCormack MK, Perlman SL, Carlozzi NE, Huntington Disease Quality of Life Site Investigators and Coordinators
Relationships among apathy, health-related quality of life, and function in Huntington's disease
Verhaeghe J, Bertoglio D, Kosten L, Thomae D, Verhoye M, Van Der Linden A, Wyffels L, Stroobants S, Wityak J, Dominguez C, Mrzljak L, Staelens S
Noninvasive relative quantification of [(11)C]ABP688 PET imaging in mice versus an input function measured over an arteriovenous shunt
Reif A, Chiki A, Ricci J, Lashuel HA
Generation of native, untagged Huntingtin Exon1 monomer and fibrils using a SUMO fusion strategy
Carlozzi NE, Boileau NR, Perlmutter JS, Chou KL, Stout JC, Paulsen JS, McCormack MK, Cella D, Nance MA, Lai JS, Dayalu P
Agreement between clinician-rated versus patient-reported outcomes in Huntington disease
Biscans A, Haraszti RA, Echeverria D, Miller R, Didiot MC, Nikan M, Roux L, Aronin N, Khvorova A
Hydrophobicity of lipid-conjugated siRNAs predicts productive loading to small extracellular vesicles
Espinoza FA, Vergara VM, Reyes D, Anderson NE, Harenski CL, Decety J, Rachakonda S, Damaraju E, Rashid B, Miller RL, Koenigs M, Kosson DS, Harenski K, Kiehl KA, Calhoun VD
Aberrant functional network connectivity in psychopathy from a large (N = 985) forensic sample
Covey DP, Dantrassy HM, Yohn SE, Castro A, Conn PJ, Mateo Y, Cheer JF
Inhibition of endocannabinoid degradation rectifies motivational and dopaminergic deficits in the Q175 mouse model of Huntington's disease
Shin B, Jung R, Oh H, Owens GE, Lee H, Kwak S, Lee R, Cotman SL, Lee JM, MacDonald ME, Song JJ, Vijayvargia R, Seong IS
Novel DNA aptamers that bind to mutant Huntingtin and modify its activity
Franich NR, Basso M, André EA, Ochaba J, Kumar A, Thein S, Fote G, Kachemov M, Lau AL, Yeung SY, Osmand A, Zeitlin SO, Ratan RR, Thompson LM, Steffan JS
Striatal mutant Huntingtin protein levels decline with age in homozygous Huntington's disease knock-in mouse models
J Huntingtons Dis
June 1, 2018
7 (2) :137-150
7 (2) :137-150
PMID: 29843246
PMCID: PMC6002862
DOI: 10.3233/JHD-170274
Suelves N, Miguez A, López-Benito S, Barriga GG, Giralt A, Alvarez-Periel E, Arévalo JC, Alberch J, Ginés S, Brito V
Early downregulation of p75(NTR) by genetic and pharmacological approaches delays the onset of motor deficits and striatal dysfunction in Huntington's disease mice
Martin DDO, Kay C, Collins JA, Nguyen YT, Slama RA, Hayden MR
A human huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington disease
Bertoglio D, Kosten L, Verhaeghe J, Thomae D, Wyffels L, Stroobants S, Wityak J, Dominguez C, Mrzljak L, Staelens S
Longitudinal characterization of mGluR5 using (11)C-ABP688 PET imaging in the Q175 mouse model of Huntington's disease
McClory H, Wang X, Sapp E, Gatune LW, Iuliano M, Wu CY, Nathwani G, Kegel-Gleason KB, DiFiglia M, Li X
The COOH-terminal domain of huntingtin interacts with RhoGEF kalirin and modulates cell survival
Schuldenzucker V, Schubert R, Muratori LM, Freisfeld F, Rieke L, Matheis T, Schramke S, Motlik J, Kemper N, Radespiel U, Reilmann R
Behavioral assessment of stress compensation in minipigs transgenic for the Huntington gene using cortisol levels: a proof-of-concept study
Chao MJ, Kim KH, Shin JW, Lucente D, Wheeler VC, Li H, Roach JC, Hood L, Wexler NS, Jardim LB, Holmans P, Jones L, Orth M, Kwak S, MacDonald ME, Gusella JF, Lee JM
Population-specific genetic modification of Huntington's disease in Venezuela
Mestre TA, Busse M, Davis AM, Quinn L, Rodrigues FB, Burgunder JM, Carlozzi NE, Walker F, Ho AK, Sampaio C, Goetz CG, Cubo E, Martinez-Martin P, Stebbins GT, Members of the MDS Committee on Rating Scales Development
Rating scales and performance-based measures for assessment of functional ability in Huntington's disease: critique and recommendations
Mov Disord Clin Pract
May 9, 2018
5 (4) :361-372
5 (4) :361-372
PMID: 30363510
PMCID: PMC6174516
DOI: 10.1002/mdc3.12617
Castro E, Polosecki P, Rish I, Pustina D, Warner JH, Wood A, Sampaio C, Cecchi GA
Baseline multimodal information predicts future motor impairment in premanifest Huntington's disease
Lee JK, Conrad A, Epping E, Mathews K, Magnotta V, Dawson JD, Nopoulos P
Effect of trinucleotide repeats in the Huntington's gene on intelligence
Pattamatta A, Cleary JD, Ranum LPW
All in the family: repeats and ALS/FTD
Gregory S, Crawford H, Seunarine K, Leavitt B, Durr A, Roos RAC, Scahill RI, Tabrizi SJ, Rees G, Langbehn D, Orth M
Natural biological variation of white matter microstructure is accentuated in Huntington's disease
Beckmann H, Bohlen S, Saft C, Hoffmann R, Gerss J, Muratori L, Ringelstein EB, Landwehrmeyer GB, Reilmann R
Objective assessment of gait and posture in premanifest and manifest Huntington disease - A multi-center study