Article
A faulty interaction between SOD1 and hCCS in neurodegenerative disease.
Scientific reports - 10 Jun 2016
Wright Gareth S A, Antonyuk Svetlana V, Hasnain S Samar
Abstract excerpt
A proportion of Amyotrophic lateral sclerosis (ALS) cases result from impaired mutant superoxide dismutase-1 (SOD1) maturation. The copper chaperone for SOD1 (hCCS) forms a transient complex with SOD1 and catalyses the final stages of its maturation. We find that a neurodegenerative disease-associated hCCS mutation abrogates the interaction with SOD1 by inhibiting hCCS zinc binding. Analogously, SOD1 zinc loss...
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