Article
Familial amyotrophic lateral sclerosis (FALS)-linked SOD1 mutation accelerates neuronal cell death by activating cleavage of caspase-4 under ER stress in an in vitro model of FALS.
Neurochemistry international - 1 Dec 2010
Koyama Yoshihisa, Hiratsuka Toru, Matsuzaki Shinsuke, Yamagishi Satoru, Kato Shinsuke, Katayama Taiichi, Tohyama Masaya
Abstract excerpt
Recently, endoplasmic reticulum (ER) dysfunction has been implicated in the pathogenesis of familial amyotrophic lateral sclerosis (FALS). Although up-regulation of caspase-12 has been reported in G93A SOD1 transgenic mice, it is controversial whether similar mechanisms operate in human FALS. We found that ER stress in cells stably expressing L84V SOD1 induces neuronal cell death and accelerates cleavage of...
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