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Article

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 - 19 Aug 2008

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

Abstract excerpt

Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disorder caused by CAG repeat expansions within the voltage-gated calcium (Ca(V)) 2.1 channel gene. It remains controversial whether the mutation exerts neurotoxicity by changing the function of Ca(V)2.1 channel or through a gain-of-function mechanism associated with accumulation of the expanded polyglutamine protein. We generated three strains of...

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Identifiers

PMID: 18687887
DOI: 10.1073/pnas.0804350105
PMCID: PMC2503926

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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