Article
Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 15 Mar 2002
Higgins Cynthia M J, Jung Cheolwha, Ding Hongliu, Xu Zuoshang
Abstract excerpt
Mutations in Cu, Zn superoxide dismutase (SOD1) cause a fraction of amyotrophic lateral sclerosis (ALS), which involves motoneuron degeneration, paralysis, and death. An acquired activity by mutant SOD1 is responsible for the cellular toxicity, but how mutant SOD1 kills motoneurons is unclear. In transgenic mouse models of ALS, mitochondrial degeneration occurs early, before disease onset, raising the question of...
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