Article
Amyotrophic lateral sclerosis: a proposed mechanism.
Proceedings of the National Academy of Sciences of the United States of America - 25 Jun 2002
Okado-Matsumoto Ayako, Fridovich Irwin
Abstract excerpt
Missense mutations in Cu,Zn-superoxide dismutase (SOD1) account for approximately 20% of familial amyotrophic lateral sclerosis (FALS) through some, as yet undefined, toxic gain of function that leads to gradual death of motor neurons. Mitochondrial swelling and vacuolization are early signs of incipient motor neuron death in FALS. We previously reported that SOD1 exists in the intermembrane space of...
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