Article
Cu,Zn-superoxide dismutase increases toxicity of mutant and zinc-deficient superoxide dismutase by enhancing protein stability.
The Journal of biological chemistry - 29 Oct 2010
Garner Mary Anne, Ricart Karina C, Roberts Blaine R, Bomben Valerie C, Basso Manuela, Ye Yaozu, Sahawneh John, Franco Maria Clara, Beckman Joseph S, Estévez Alvaro G
Abstract excerpt
When replete with zinc and copper, amyotrophic lateral sclerosis (ALS)-associated mutant SOD proteins can protect motor neurons in culture from trophic factor deprivation as efficiently as wild-type SOD. However, the removal of zinc from either mutant or wild-type SOD results in apoptosis of motor neurons through a copper- and peroxynitrite-dependent mechanism. It has also been shown that motor neurons isolated...
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