The role of ER stress for the pathogenesis of SCA3 in primary cell culture experiment

The spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, is an autosomal dominantly inherited neurodegenerative disorder, which is caused by an expanded CAG repeat in the ataxin-3 gene. After a decade of research the function of ataxin-3 is still unidentified. Here we investigated, whether there is a causal relationship between the pathogenesis of SCA3 and endoplasmic reticulum (ER) stress. Therefore we analyzed mouse embryonic fibroblasts (Mef's), which derived from SCA3 mutant mice, generated by a gene trap (gt) approach. Heterozygous (wt/gt) and homozygous (gt/gt) mutant Mef's for SCA3…