Article
Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS.
Progress in neurobiology - 1 May 2008
Turner Bradley J, Talbot Kevin
Abstract excerpt
Gain-of-function mutations in the Cu,Zn-superoxide dismutase (SOD1) gene are implicated in progressive motor neuron death and paralysis in one form of inherited amyotrophic lateral sclerosis (ALS). At present, transgenic expression of 12 human SOD1 mutations driven by the endogenous promoter is disease-causative and uniformly lethal in mice and rats, despite tremendous biochemical and biophysical variation...
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