Abnormal features in mutant cerebellar Purkinje cells lacking junctophilins.

Abstract:

:Junctional membrane complexes (JMCs) generated by junctophilins are required for Ca(2+)-mediated communication between cell-surface and intracellular channels in excitable cells. Knockout mice lacking neural junctophilins (JP-DKO) show severe motor defects and irregular cerebellar plasticity due to abolished channel crosstalk in Purkinje cells (PCs). To precisely understand aberrations in JP-DKO mice, we further analyzed the mutant PCs. During the induction of cerebellar plasticity via electrical stimuli, JP-DKO PCs showed insufficient depolarizing responses. Immunochemistry detected mild impairment in synaptic maturation and hyperphosphorylation of protein kinase Cgamma in JP-DKO PCs. Moreover, gene expression was slightly altered in the JP-DKO cerebellum. Therefore, the mutant PCs bear marginal but widespread abnormalities, all of which likely cause cerebellar motor defects in JP-DKO mice.

authors

Ikeda A,Miyazaki T,Kakizawa S,Okuno Y,Tsuchiya S,Myomoto A,Saito SY,Yamamoto T,Yamazaki T,Iino M,Tsujimoto G,Watanabe M,Takeshima H

doi

10.1016/j.bbrc.2007.09.062

subject

Has Abstract

pub_date

2007-11-23 00:00:00

pages

835-9

issue

3

eissn

0006-291X

issn

1090-2104

pii

S0006-291X(07)02031-1

journal_volume

363

pub_type

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