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Preventing polyglutamine-induced activation of c-Jun delays neuronal dysfunction in a mouse model of SCA7 retinopathy.

Abstract：

:We have approached the role of cellular stress in neurodegenerative diseases caused by polyglutamine expansion (polyQ) in the context of Spinocerebellar ataxia type 7 (SCA7) that includes retinal degeneration. Using the R7E mouse, in which polyQ-ataxin-7 is specifically over-expressed in rod photoreceptors, we previously showed that rod dysfunction correlated to moderate and prolonged activation of the JNK/c-Jun stress pathway. SCA7 retinopathy was also associated with reduced expression of rod-specific genes, including the transcription factor Nrl, which is essential for rod differentiation and function. Here, we report that R7E retinopathy is improved upon breeding with the JunAA knock-in mice, in which JNK-mediated activation of c-Jun is compromised. Expression of Nrl and its downstream targets, which are involved in phototranduction, are partially restored in the JunAA-R7E mice. We further show that c-Jun can directly repress the transcription of Nrl. Our studies suggest that polyQ-induced cellular stress leads to repression of genes necessary for neuronal fate and function.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Merienne K,Friedman J,Akimoto M,Abou-Sleymane G,Weber C,Swaroop A,Trottier Y

doi

10.1016/j.nbd.2006.11.002

subject

Has Abstract

pub_date

2007-03-01 00:00:00

pages

571-81

issue

3

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(06)00292-0

journal_volume

25

pub_type

杂志文章

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