Article
Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice.
Neurobiology of disease - 1 Jul 1998
Borchelt D R, Wong P C, Becher M W, Pardo C A, Lee M K, Xu Z S, Thinakaran G, Jenkins N A, Copeland N G, Sisodia S S, Cleveland D W, Price D L, Hoffman P N
Abstract excerpt
Superoxide dismutase 1 (SOD1), a ubiquitously expressed enzyme, detoxifies superoxide radicals and participates in copper homeostasis. Mutations in this enzyme have been linked to a subset of autosomal dominant cases of familial amyotrophic lateral sclerosis (FALS), a disorder characterized by selective degeneration of motor neurons. Transgenic mice expressing FALS mutant human (Hu) SOD1 at high levels develop a...
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