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Publication : Abnormal features in mutant cerebellar Purkinje cells lacking junctophilins.

First Author  Ikeda A Year  2007
Journal  Biochem Biophys Res Commun Volume  363
Issue  3 Pages  835-9
PubMed ID  17904530 Mgi Jnum  J:127350
Mgi Id  MGI:3763599 Doi  10.1016/j.bbrc.2007.09.062
Citation  Ikeda A, et al. (2007) Abnormal features in mutant cerebellar Purkinje cells lacking junctophilins. Biochem Biophys Res Commun 363(3):835-9
abstractText  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.
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