Article
Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo.
Neurobiology of disease - 1 Apr 2009
Son Marjatta, Fu Qiao, Puttaparthi Krishna, Matthews Christina M, Elliott Jeffrey L
Abstract excerpt
Over-expression of CCS in G93A SOD1 mice accelerates neurological disease and enhances mitochondrial pathology. We studied the effect of CCS over-expression in transgenic mice expressing G37R, G86R or L126Z SOD1 mutations in order to understand factors which influence mitochondrial dysfunction. Over-expression of CCS markedly decreased survival and produced mitochondrial vacuolation in G37R SOD1 mice but not in...
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