Article
MnSOD deficiency has a differential effect on disease progression in two different ALS mutant mouse models.
Muscle & nerve - 1 Sept 2008
Muller Florian L, Liu Yuhong, Jernigan Amanda, Borchelt David, Richardson Arlan, Van Remmen Holly
Abstract excerpt
Mitochondrial dysfunction and oxidative stress are thought to participate in the pathogenesis of amyotrophic lateral sclerosis (ALS). The purpose of this study was to determine the effect of reduced mitochondrial antioxidant defense on lifespan and disease progression in two mouse models of familial ALS (G93A and H46R/H48Q mutant lines) that represent pseudo-wildtype and metal-deficient ALS mutants, respectively....
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