Article
Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis.
Journal of neurochemistry - 1 Apr 2004
Takeuchi Hideyuki, Niwa Jun-ichi, Hishikawa Nozomi, Ishigaki Shinsuke, Tanaka Fumiaki, Doyu Manabu, Sobue Gen
Abstract excerpt
Abstract Dorfin is a RING-finger type ubiquitin ligase for mutant superoxide dismutase 1 (SOD1) that enhances its degradation. Mutant SOD1s cause familial amyotrophic lateral sclerosis (FALS) through the gain of unelucidated toxic properties. We previously showed that the accumulation of mutant SOD1 in the mitochondria triggered the release of cytochrome c, followed by the activation of the caspase cascade and...
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