Article
Copper(2+) binding to the surface residue cysteine 111 of His46Arg human copper-zinc superoxide dismutase, a familial amyotrophic lateral sclerosis mutant.
Biochemistry - 18 Jul 2000
Liu H, Zhu H, Eggers D K, Nersissian A M, Faull K F, Goto J J, Ai J, Sanders-Loehr J, Gralla E B, Valentine J S
Abstract excerpt
Mutations in copper-zinc superoxide dismutase (CuZnSOD) cause 25% of familial amyotrophic lateral sclerosis (FALS) cases. This paper examines one such mutant, H46R, which has no superoxide dismutase activity yet presumably retains the gain-of-function activity that leads to disease. We demonstrate that Cu(2+) does not bind to the copper-specific catalytic site of H46R CuZnSOD and that Cu(2+) competes with other...
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