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Activation of mutant protein kinase Cgamma leads to aberrant sequestration and impairment of its cellular function.

Abstract：

:Mutations in protein kinase Cgamma (PKCgamma) cause the neurodegenerative disease spinocerebellar ataxia type 14 (SCA14). In this study, expression of an extensive panel of known SCA14-associated PKCgamma mutations as fusion proteins in cell culture led to the consistent formation of cytoplasmic aggregates in response to purinoceptor stimulation. Aggregates co-stained with antibodies to phosphorylated PKCgamma and the early endosome marker EEA1 but failed to redistribute to the cell membrane under conditions of oxidative stress. These studies suggest that Purkinje cell damage in SCA14 may result from a reduction of PKCgamma activity due its aberrant sequestration in the early endosome compartment.

journal_name

Biochem Biophys Res Commun

journal_title

Biochemical and biophysical research communications

authors

Doran G,Davies KE,Talbot K

doi

10.1016/j.bbrc.2008.05.072

subject

Has Abstract

pub_date

2008-08-01 00:00:00

pages

447-53

issue

3

eissn

0006-291X

issn

1090-2104

pii

S0006-291X(08)00957-1

journal_volume

372

pub_type

杂志文章

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