Scientific Publications

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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Wild EJ, Boggio R, Langbehn D, Robertson N, Haider S, Miller JR, Zetterberg H, Leavitt BR, Kuhn R, Tabrizi SJ, Macdonald D, Weiss A
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington`s disease patients
J Clin Invest
May 1, 2015
125 (5) :1979-86
PMID: 25844897
PMCID: PMC4463213
Novak MJ, Seunarine KK, Gibbard CR, McColgan P, Draganski B, Friston K, Clark CA, Tabrizi SJ
Basal ganglia-cortical structural connectivity in Huntington`s disease
Hum Brain Mapp
May 1, 2015
36 (5) :1728-40
PMID: 25640796
Jin J, Peng Q, Hou Z, Jiang M, Wang X, Langseth AJ, Tao M, Barker PB, Mori S, Bergles DE, Ross CA, Detloff PJ, Zhang J, Duan W
Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington`s disease
Hum Mol Genet
May 1, 2015
24 (9) :2508-27
PMID: 25609071
PMCID: PMC4383863
Biagioli M, Ferrari F, Mendenhall EM, Zhang Y, Erdin S, Vijayvargia R, Vallabh SM, Solomos N, Manavalan P, Ragavendran A, Ozsolak F, Lee JM, Talkowski ME, Gusella JF, Macdonald ME, Park PJ, Seong IS
Htt CAG repeat expansion confers pleiotropic gains of mutant huntingtin function in chromatin regulation
Hum Mol Genet
May 1, 2015
24 (9) :2442-57
PMID: 25574027
PMCID: 4383859
Wityak J, McGee KF, Conlon MP, Song RH, Duffy BC, Clayton B, Lynch M, Wang G, Freeman E, Haber J, Kitchen DB, Manning DD, Ismail J, Khmelnitsky Y, Michels P, Webster J, Irigoyen M, Luche M, Hultman M, Bai M, Kuok ID, Newell R, Lamers M, Leonard P, Yates D, Matthews K, Ongeri L, Clifton S, Mead T, Deupree S, Wheelan P, Lyons K, Wilson C, Kiselyov A, Toledo-Sherman L, Beconi M, Muñoz-Sanjuan I, Bard J, Dominguez C
Lead optimization toward proof-of-concept tools for Huntington's disease within a 4-(1H-pyrazol-4-yl) pyrimidine class of pan-JNK inhibitors
J Med Chem
April 9, 2015
58 (7) :2967-87
PMID: 25760409
Larson E, Fyfe I, Morton AJ, Monckton DG
Age-, tissue- and length-dependent bidirectional somatic CAG•CTG repeat instability in an allelic series of R6/2 Huntington disease mice
Neurobiol Dis
April 1, 2015
76 :98-111
PMID: 25662336
Sanders SS, Hayden MR
Aberrant palmitoylation in Huntington disease
Biochem Soc Trans
April 1, 2015
43 (2) :205-10
PMID: 25849918
Hering T, Birth N, Taanman JW, Orth M
Selective striatal mtDNA depletion in end-stage Huntington`s disease R6/2 mice
Exp Neurol
April 1, 2015
266 :22-9
PMID: 25682918
Khakh BS, McCarthy KD
Astrocyte calcium signaling: from observations to functions and the challenges therein
Cold Spring Harb Perspect Biol
April 1, 2015
7 (4) :a020404
PMID: 25605709
Valenza M, Marullo M, Di Paolo E, Cesana E, Zuccato C, Biella G, Cattaneo E
Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington`s disease
Cell Death Differ
April 1, 2015
22 (4) :690-702
PMID: 25301063
PMCID: 4356339
Wassef SN, Wemmie J, Johnson CP, Johnson H, Paulsen JS, Long JD, Magnotta VA
T1rho imaging in premanifest Huntington disease reveals changes associated with disease progression
Mov Disord
March 29, 2015
30 (8) :1107-14
PMID: 25820773
Orsatti L, Speziale R, Orsale MV, Caretti F, Veneziano M, Zini M, Monteagudo E, Lyons K, Beconi M, Chan K, Herbst T, Toledo-Sherman L, Munoz-Sanjuan I, Bonelli F, Dominguez C
A single-run liquid chromatography mass spectrometry method to quantify neuroactive kynurenine pathway metabolites in rat plasma
J Pharm Biomed Anal
March 25, 2015
107 :426-31
PMID: 25668794
Huang B, Lucas T, Kueppers C, Dong X, Krause M, Bepperling A, Buchner J, Voshol H, Weiss A, Gerrits B, Kochanek S
Scalable production in human cells and biochemical characterization of full-length normal and mutant huntingtin
PLoS One
March 23, 2015
10 (3) :e0121055
PMID: 25799558
PMCID: 4370734
Penrose SD, Stott AJ, Breccia P, Haughan AF, Burli RW, Jarvis RE, Dominguez C
Inter- and intramolecular annulation strategies to a cyclopentanone building block containing an all-carbon quaternary stereogenic center
Org Lett
March 20, 2015
17 (6) :1401-4
PMID: 25720587
Cheng HM, Chern Y, Chen IH, Liu CR, Li SH, Chun SJ, Rigo F, Bennett CF, Deng N, Feng Y, Lin CS, Yan YT, Cohen SN, Cheng TH
Effects on murine behavior and lifespan of selectively decreasing expression of mutant huntingtin allele by supt4h knockdown
PLoS Genet
March 11, 2015
11 (3) :e1005043
PMID: 25760041
PMCID: 4356588
Estrada-Sanchez AM, Burroughs CL, Cavaliere S, Barton SJ, Chen S, Yang XW, Rebec GV
Cortical efferents lacking mutant huntingtin improve striatal neuronal activity and behavior in a conditional mouse model of Huntington`s disease
J Neurosci
March 11, 2015
35 (10) :4440-51
PMID: 25762686
PMCID: 4355206
Sturrock A, Laule C, Wyper K, Milner RA, Decolongon J, Dar Santos R, Coleman AJ, Carter K, Creighton S, Bechtel N, Bohlen S, Reilmann R, Johnson HJ, Hayden MR, Tabrizi SJ, Mackay AL, Leavitt BR
A longitudinal study of magnetic resonance spectroscopy Huntington`s disease biomarkers
Mov Disord
March 1, 2015
30 (3) :393-401
PMID: 25690257
Cao Y, Bartolomé-Martín D, Rotem N, Rozas C, Dellal SS, Chacon MA, Kadriu B, Gulinello M, Khodakhah K, Faber DS
Rescue of homeostatic regulation of striatal excitability and locomotor activity in a mouse model of Huntington's disease
Proc Natl Acad Sci USA
February 17, 2015
112 (7) :2239-44
PMID: 25646456
PMCID: PMC4343133
Toledo-Sherman LM, Prime ME, Mrzljak L, Beconi MG, Beresford A, Brookfield FA, Brown CJ, Cardaun I, Courtney SM, Dijkman U, Hamelin-Flegg E, Johnson PD, Kempf V, Lyons K, Matthews K, Mitchell WL, O`Connell C, Pena P, Powell K, Rassoulpour A, Reed L, Reindl W, Selvaratnam S, Friley WW, Weddell DA, Went NE, Wheelan P, Winkler C, Winkler D, Wityak J, Yarnold CJ, Yates D, Munoz-Sanjuan I, Dominguez C
Development of a series of aryl pyrimidine kynurenine monooxygenase inhibitors as potential therapeutic agents for the treatment of Huntington`s disease
J Med Chem
February 12, 2015
58 (3) :1159-83
PMID: 25590515
Poudel GR, Stout JC, Dominguez DJ F, Churchyard A, Chua P, Egan GF, Georgiou-Karistianis N
Longitudinal change in white matter microstructure in Huntington`s disease: The IMAGE-HD study
Neurobiol Dis
February 1, 2015
74 :406-12
PMID: 25497085
Dietz KN, Di Stefano L, Maher RC, Zhu H, Macdonald ME, Gusella JF, Walker JA
The Drosophila Huntington`s disease gene ortholog dhtt influences chromatin regulation during development
Hum Mol Genet
January 15, 2015
24 (2) :330-45
PMID: 25168387
Nagy D, Tingley FD 3rd, Stoiljkovic M, Hajós M
Application of neurophysiological biomarkers for Huntington's disease: evaluating a phosphodiesterase 9A inhibitor
Exp Neurol
January 1, 2015
263 :122-31
PMID: 25315303
Curtin PC, Farrar AM, Oakeshott S, Sutphen J, Berger J, Mazzella M, Cox K, He D, Alosio W, Park LC, Howland D, Brunner D
Cognitive training at a young age attenuates deficits in the zQ175 mouse model of HD
Front Behav Neurosci
January 1, 2015
9 :361
PMID: 26793080
PMCID: PMC4707270
Alterman JF, Hall LM, Coles AH, Hassler MR, Didiot MC, Chase K, Abraham J, Sottosanti E, Johnson E, Sapp E, Osborn MF, Difiglia M, Aronin N, Khvorova A
Hydrophobically modified siRNAs silence huntingtin mRNA in primary neurons and mouse brain
Mol Ther Nucleic Acids
January 1, 2015
4 :e266
PMID: 26623938
Carroll JB, Bates GP, Steffan J, Saft C, Tabrizi SJ
Treating the whole body in Huntington`s disease
The Lancet Neurology
January 1, 2015
14 (11) :1135-1142
PMID: 26466780
Oosterloo M, Van Belzen MJ, Bijlsma EK, Roos RA
Is there convincing evidence that intermediate repeats in the HTT gene cause Huntington`s disease?
J Huntingtons Dis
January 1, 2015
4 (2) :141-8
PMID: 26397895
Wood NI, Morton AJ
Social behaviour is impaired in the R6/2 mouse model of Huntington's disease
J Huntingtons Dis
January 1, 2015
4 (1) :61-73
PMID: 26333258
Reilmann R, Lippross V, Holzner E, Gigengack F, Bohlen S, Kugel H, Deppe M, Osada N, Lucke M, Riess O, Nguyen HP, Von Horsten S, Schafers K, Schafers M, Jacobs AH, Hermann S
FDG uPET fails to detect a disease-specific phenotype in rats transgenic for Huntington's disease
J Huntingtons Dis
January 1, 2015
4 (1) :37-47
PMID: 26333256
Musso M, Westervelt HJ, Long JD, Morgan E, Woods SP, Smith MM, Lu W, Paulsen JS, Predict-HD Investigators of the Huntington Study Group
Intra-individual variability in prodromal Huntington disease and its relationship to genetic burden
J Int Neuropsychol Soc
January 1, 2015
21 (1) :44064
PMID: 26304055
PMCID: PMC4549971
Carroll JB, Deik A, Fossale E, Weston RM, Guide JR, Arjomand J, Kwak S, Clish CB, MacDonald ME
HdhQ111 mice exhibit tissue specific metabolite profiles that include striatal lipid accumulation
PLoS One
January 1, 2015
10 (8) :e0134465
PMID: 26295712
PMCID: PMC4546654
Odish OF, Caeyenberghs K, Hosseini H, van den Bogaard SJ, Roos RA, Leemans A
Dynamics of the connectome in Huntington`s disease: A longitudinal diffusion MRI study
Neuroimage Clin
January 1, 2015
9 :32-43
PMID: 26288754
PMCID: PMC4536305
Grondin R, Ge P, Chen Q, Sutherland JE, Zhang Z, Gash DM, Stiles DK, Stewart GR, Sah DW, Kaemmerer WF
Onset time and durability of Huntingtin suppression in Rhesus putamen after direct infusion of Antihuntingtin siRNA
Mol Ther Nucleic Acids
January 1, 2015
4 :e245
PMID: 26125484
PMCID: PMC4561652
Naseri NN, Xu H, Bonica J, Vonsattel JP, Cortes EP, Park LC, Arjomand J, Gibson GE
Abnormalities in the tricarboxylic Acid cycle in Huntington disease and in a Huntington disease mouse model
J Neuropathol Exp Neurol
January 1, 2015
74 (6) :527-37
PMID: 25978848
PMCID: PMC4435838
Carty N, Berson N, Tillack K, Thiede C, Scholz D, Kottig K, Sedaghat Y, Gabrysiak C, Yohrling G, von der Kammer H, Ebneth A, Mack V, Munoz-Sanjuan I, Kwak S
Characterization of HTT inclusion size, location, and timing in the zQ175 mouse model of Huntingtons disease: an in vivo high-content imaging study
PLoS One
January 1, 2015
10 (4) :e0123527
PMID: 25859666
PMCID: 4393127
Callaghan J, Stopford C, Arran N, Boisse MF, Coleman A, Santos RD, Dumas EM, Hart EP, Justo D, Owen G, Read J, Say MJ, Durr A, Leavitt BR, Roos RA, Tabrizi SJ, Bachoud-Levi AC, Bourdet C, van Duijn E, Craufurd D
Reliability and factor structure of the Short Problem Behaviors Assessment for Huntington`s disease (PBA-s) in the TRACK-HD and REGISTRY studies
J Neuropsychiatry Clin Neurosci
January 1, 2015
27 (1) :59-64
PMID: 25716488
Gabery S, Georgiou-Karistianis N, Lundh SH, Cheong RY, Churchyard A, Chua P, Stout JC, Egan GF, Kirik D, Petersen A
Volumetric analysis of the hypothalamus in Huntington Disease using 3T MRI: the IMAGE-HD Study
PLoS One
January 1, 2015
10 (2) :e0117593
PMID: 25659157
PMCID: 4319930
Cummings J, Mintzer J, Brodaty H, Sano M, Banerjee S, Devanand DP, Gauthier S, Howard R, Lanctot K, Lyketsos CG, Peskind E, Porsteinsson AP, Reich E, Sampaio C, Steffens D, Wortmann M, Zhong K, International Psychogeriatric Association
Agitation in cognitive disorders: International Psychogeriatric Association provisional consensus clinical and research definition
Int Psychogeriatr
January 1, 2015
27 (1) :44029
PMID: 25311499
PMCID: PMC4301197
Odish OF, van den Berg-Huysmans AA, van den Bogaard SJ, Dumas EM, Hart EP, Rombouts SA, van der Grond J, Roos RA, Track-Hd Investigator Group
Longitudinal resting state fMRI analysis in healthy controls and premanifest Huntington`s disease gene carriers: a three-year follow-up study
Hum Brain Mapp
January 1, 2015
36 (1) :110-9
PMID: 25139578
Mrzljak L, Munoz-Sanjuan I
Therapeutic strategies for Huntington`s Disease
Curr Top Behav Neurosci
January 1, 2015
22 :161-201
PMID: 24277342
Poudel GR, Stout JC, Dominguez DJ, Gray MA, Salmon L, Churchyard A, Chua P, Borowsky B, Egan GF, Georgiou-Karistianis N
Functional changes during working memory in Huntington`s disease: 30-month longitudinal data from the IMAGE-HD study
Brain Struct Funct
January 1, 2015
220 (1) :501-12
PMID: 24240602