[HTML][HTML] Proteasomal degradation of mutant superoxide dismutases linked to amyotrophic lateral sclerosis
L Di Noto, LJ Whitson, X Cao, PJ Hart… - Journal of Biological …, 2005 - ASBMB
Mutations in copper-zinc superoxide dismutase cause the neurodegenerative disease
amyotrophic lateral sclerosis. Many of the mutant proteins have increased turnover in vivo
and decreased thermal stability. Here we show that purified, metal-free superoxide
dismutases are degraded in vitro by purified 20 S proteasome in the absence of ATP and
without ubiquitinylation, whereas their metal-bound counterparts are not. The rate of
degradation by the proteasome varied among the mutants studied, and the rate correlated …
amyotrophic lateral sclerosis. Many of the mutant proteins have increased turnover in vivo
and decreased thermal stability. Here we show that purified, metal-free superoxide
dismutases are degraded in vitro by purified 20 S proteasome in the absence of ATP and
without ubiquitinylation, whereas their metal-bound counterparts are not. The rate of
degradation by the proteasome varied among the mutants studied, and the rate correlated …