Article
Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models.
Proceedings of the National Academy of Sciences of the United States of America - 28 Aug 2007
Zetterström Per, Stewart Heather G, Bergemalm Daniel, Jonsson P Andreas, Graffmo Karin S, Andersen Peter M, Brännström Thomas, Oliveberg Mikael, Marklund Stefan L
Abstract excerpt
Mutants of superoxide dismutase-1 (SOD1) cause ALS by an unidentified cytotoxic mechanism. We have previously shown that the stable SOD1 mutants D90A and G93A are abundant and show the highest levels in liver and kidney in transgenic murine ALS models, whereas the unstable G85R and G127X mutants are scarce but enriched in the CNS. These data indicated that minute amounts of misfolded SOD1 enriched in the motor...
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