DRPLA transgenic mouse substrains carrying single copy of full-length mutant human DRPLA gene with variable sizes of expanded CAG repeats exhibit CAG repeat length- and age-dependent changes in behavioral abnormalities and gene expression profiles

Neurobiology of Disease

Volumn 46, Issue 2, 2012, Pages 336-350

  Suzuki, Kazushi ^a   Zhou, Jiayi ^a,f   Sato, Toshiya ^b   Takao, Keizo ^c,d,e   Miyagawa, Tsuyoshi ^c,d,e   Oyake, Mutsuo ^b,g   Yamada, Mitunori ^b   Takahashi, Hitoshi ^b   Takahashi, Yuji ^a   Goto, Jun ^a   Tsuji, Shoji ^a  

^a UNIVERSITY OF TOKYO   (Japan)

^b NIIGATA UNIVERSITY   (Japan)

^c NATIONAL INSTITUTE FOR PHYSIOLOGICAL SCIENCES   (Japan)

^d KYOTO UNIVERSITY   (Japan)

^e FUJITA HEALTH UNIVERSITY   (Japan)

^f BOSTON UNIVERSITY   (United States)

^g NAGAOKA RED CROSS HOSPITAL   (Japan)

Author keywords

Behavioral abnormality; Calcium signaling; Dentatorubral pallidoluysian atrophy; DRPLA transgenic mice; Expression profiling; Microarray; Neurodegeneration; Neuropeptides; Polyglutamine; Transcriptional dysregulation

Indexed keywords

DRPLA PROTEIN; MUTANT PROTEIN; NEUROPEPTIDE; NUCLEAR PROTEIN; UNCLASSIFIED DRUG;

AGE; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BEHAVIOR DISORDER; CAG REPEAT; CALCIUM SIGNALING; CELL NUCLEUS; CEREBELLUM; CLUSTER ANALYSIS; CONTROLLED STUDY; DENTATORUBROPALLIDOLUYSIAN ATROPHY; DISEASE SEVERITY; DOWN REGULATION; DRPLA GENE; GENE; GENE CLUSTER; GENE EXPRESSION PROFILING; MEMORY DISORDER; MICROARRAY ANALYSIS; MOTOR DYSFUNCTION; MOUSE; NONHUMAN; PRIORITY JOURNAL; REFERENCE MEMORY; SPATIAL MEMORY; TRANSGENIC MOUSE; UPREGULATION;

AGE FACTORS; ANIMALS; HUMANS; MAZE LEARNING; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; MOTOR ACTIVITY; MUTATION; NERVE TISSUE PROTEINS; PROTEIN BINDING; TRANSCRIPTOME; TRINUCLEOTIDE REPEAT EXPANSION;

EID: 84862804974     PISSN: 09699961     EISSN: 1095953X     Source Type: Journal    
DOI: 10.1016/j.nbd.2012.01.014     Document Type: Article

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