Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels

Proceedings of the National Academy of Sciences of the United States of America

Volumn 105, Issue 33, 2008, Pages 11987-11992

  Watase, Kei ^a,b   Barrett, Curtis F ^c,f   Miyazaki, Taisuke ^d   Ishiguro, Taro ^a   Ishikawa, Kinya ^a   Hu, Yuanxin ^e   Unno, Toshinori ^a   Sun, Yaling ^b   Kasai, Sayumi ^a   Watanabe, Masahiko ^d   Gomez, Christopher M ^e   Mizusawa, Hidehiro ^a   Tsien, Richard W ^c   Zoghbi, Huda Y ^b  

^a TOKYO MEDICAL AND DENTAL UNIVERSITY   (Japan)

^b BAYLOR COLLEGE OF MEDICINE   (United States)

^c STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d HOKKAIDO UNIVERSITY   (Japan)

^e UNIVERSITY OF CHICAGO   (United States)

^f LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

Cerebullum; P Q type voltage dependent calcium channel; Polyglutamine

Indexed keywords

ATAXIN 6; CYSTEINE PROTEINASE; POLYGLUTAMINE; PROTEIN CACNA1A; PROTEIN SUBUNIT; UNCLASSIFIED DRUG; VOLTAGE GATED CALCIUM CHANNEL; VOLTAGE GATED CALCIUM CHANNEL 2.1;

ANIMAL TISSUE; ARTICLE; CALCIUM CURRENT; CEREBELLUM; CONTROLLED STUDY; DISEASE COURSE; ELECTROPHYSIOLOGY; EXON; GENE LOCUS; GENE SEQUENCE; GENE TARGETING; IMMUNOREACTIVITY; MOTOR DYSFUNCTION; MOUSE; NERVE CELL; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN PROCESSING; PURKINJE CELL; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; RNA SPLICING;

AGING; ALTERNATIVE SPLICING; ANIMALS; CALCIUM CHANNELS, N-TYPE; DISEASE PROGRESSION; ELECTROPHYSIOLOGY; EXONS; GENE EXPRESSION; MICE; MICE, TRANSGENIC; MUTANT PROTEINS; MUTATION; NERVOUS SYSTEM DISEASES; PHENOTYPE; SPINOCEREBELLAR ATAXIAS; TRANSGENES;

MUS;

EID: 50149093030     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.0804350105     Document Type: Article

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