Article
SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter.
Nature neuroscience - 1 May 1999
Trotti D, Rolfs A, Danbolt N C, Brown R H, Hediger M A
Abstract excerpt
The mechanism by which Cu2+/Zn2+ superoxide dismutase (SOD1) mutants lead to motor neuron degeneration in familial amyotrophic lateral sclerosis (FALS) is unknown. We show that oxidative reactions triggered by hydrogen peroxide and catalyzed by A4V and I113T mutant but not wild-type SOD1 inactivated the glutamate transporter human GLT1. Chelation of the copper ion of the prosthetic group of A4V prevented GLT1...
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