Infant and adult SCA13 mutations differentially affect Purkinje cell excitability, maturation, and viability in vivo.

ABSTRACT: Mutations in KCNC3, which encodes the Kv3.3 K⁺ channel, cause spinocerebellar ataxia 13 (SCA13). SCA13 exists in distinct forms with onset in infancy or adulthood. Using zebrafish, we tested the hypothesis that infant- and adult-onset mutations differentially affect the excitability and viability of Purkinje cells in vivo during cerebellar development. An infant-onset mutation dramatically and transiently increased Purkinje cell excitability, stunted process extension, impaired dendritic branching and synaptogenesis, and caused rapid cell death during cerebellar development. Reducing excitability increased early Purkinje cell survival. In contrast, an adult-onset mutation did not significantly alter basal tonic firing in Purkinje cells, but reduced excitability during evo

SUBMITTER: Hsieh JY 

PROVIDER: S-EPMC7386905 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature