Article
Misfolded CuZnSOD and amyotrophic lateral sclerosis.
Proceedings of the National Academy of Sciences of the United States of America - 1 Apr 2003
Valentine Joan Selverstone, Hart P John
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of motor neurons. The inherited form of the disease, familial ALS, represents 5-10% of the total cases, and the best documented of these are due to lesions in SOD1, the gene encoding copper-zinc superoxide dismutase (CuZnSOD). The mechanism by which mutations in SOD1 cause familial ALS is currently unknown. Two hypotheses have dominated...
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