Article
Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 19 Apr 2006
Bergemalm Daniel, Jonsson P Andreas, Graffmo Karin S, Andersen Peter M, Brännström Thomas, Rehnmark Anna, Marklund Stefan L
Abstract excerpt
Mutants of human superoxide dismutase-1 (hSOD1) cause amyotrophic lateral sclerosis (ALS), and mitochondria are thought to be primary targets of the cytotoxic action. The high expression rates of hSOD1s in transgenic ALS models give high levels of the stable mutants G93A and D90A as well as the w...
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