Article
Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS.
Journal of neuropathology and experimental neurology - 1 May 1997
Durham H D, Roy J, Dong L, Figlewicz D A
Abstract excerpt
Mutations in the Cu/Zn-superoxide dismutase (SOD-1) gene underlie some familial cases of amyotrophic lateral sclerosis (FALS), a neurodegenerative disorder characterized by loss of cortical, brainstem, and spinal motor neurons. To investigate the mechanisms responsible for the toxicity of mutant enzyme, SOD-1 cDNAs bearing mutations found in FALS patients (mSOD) were expressed in cultured spinal motor neurons,...
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