Article
Biochemical properties and in vivo effects of the SOD1 zinc-binding site mutant (H80G).
Journal of neurochemistry - 1 Sept 2011
Son Marjatta, Srikanth Uma, Puttaparthi Krishna, Luther Christina, Elliott Jeffrey L
Abstract excerpt
This study presents the initial characterization of transgenic mice with mutations in a primary zinc-binding residue (H80), either alone or with a G93A mutation. H80G;G93A superoxide dismutase 1 (SOD1) transgenic mice developed paralysis with motor neuron loss, and ubiquitin inclusion-type rather than mitochondrial vacuolar pathology. Unlike G93A SOD1-related disease, the course was not accelerated by...
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