Article
CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsp/Hsc70.
Journal of neurochemistry - 1 Jul 2004
Urushitani Makoto, Kurisu Junko, Tateno Minako, Hatakeyama Shigetsugu, Nakayama Kei-Ichi, Kato Shinsuke, Takahashi Ryosuke
Abstract excerpt
Over 100 mutants in superoxide dismutase 1 (SOD1) are reported in familial amyotrophic lateral sclerosis (ALS). However, the precise mechanism by which they are degraded through a ubiquitin-proteasomal pathway (UPP) remains unclear. Here, we report that heat-shock protein (Hsp) or heat-shock cognate (Hsc)70, and the carboxyl terminus of the Hsc70-interacting protein (CHIP), are involved in proteasomal degradation...
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