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Expression of X-chromosome linked inhibitor of apoptosis protein in mature Purkinje cells and in retinal bipolar cells in transgenic mice induces neurodegeneration.

Abstract：

:Transgenic mice with overexpression of the caspase-inhibitor, X-chromosome-linked inhibitor of apoptosis protein (XIAP) in Purkinje cell (PC) and in retinal bipolar cells (RBCs) were produced to study the regulation of cell death. Unexpectedly, an increased neurodegeneration was observed in the PCs in these L7-XIAP mice after the third postnatal week with the mice exhibiting severe ataxia. The loss of PCs was independent of Bax as shown by crossing the L7-XIAP mice with Bax gene-deleted mice. Electron microscopy revealed intact organelles in PCs but with the stacking of ER cisterns indicative of cell stress. Immunostaining for cell death proteins showed an increased phosphorylation of c-Jun in the PCs, suggesting an involvement in cell degeneration. Apart from PCs, the number of RBCs was decreased in adult retina in line with the expression pattern for the L7 promoter. The data show that overexpression of the anti-apoptotic protein XIAP in vulnerable neurons leads to enhanced cell death. The mechanisms underlying this neurodegeneration can be related to the effects of XIAP on cell stress and altered cell signaling.

journal_name

Neuroscience

journal_title

Neuroscience

authors

Korhonen L,Hansson I,Maugras C,Wehrle R,Kairisalo M,Borgkvist A,Jokitalo E,Sotelo C,Fisone G,Dusart I,Lindholm D

doi

10.1016/j.neuroscience.2008.08.005

subject

Has Abstract

pub_date

2008-10-15 00:00:00

pages

515-26

issue

3

eissn

0306-4522

issn

1873-7544

pii

S0306-4522(08)01181-0

journal_volume

156

pub_type

杂志文章

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