Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis.
Authors Hayward Lawrence J; Rodriguez Jorge A; Kim Ji W; Tiwari Ashutosh; Goto Joy J; Cabelli Diane E; Valentine Joan Selverstone; Brown Robert H Jr;
JournalJ Biol Chem
PubMed ID11854284
Over 90 different mutations in the gene encoding copper/zinc superoxide dismutase (SOD1) cause approximately 2% of amyotrophic lateral sclerosis (ALS) cases by an unknown mechanism. We engineered 14 different human ALS-related SOD1 mutants and obtained high yields of biologically metallated proteins from an Sf21 insect cell expression system. Both the ... More