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Article

Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading.

Nature neuroscience - 1 Apr 2002

Subramaniam Jamuna R, Lyons W Ernest, Liu Jian, Bartnikas Thomas B, Rothstein Jeffrey, Price Donald L, Cleveland Don W, Gitlin Jonathan D, Wong Philip C

Abstract excerpt

Copper-mediated oxidative damage is proposed to play a critical role in the pathogenesis of Cu/Zn superoxide dismutase (SOD1)-linked familial amyotrophic lateral sclerosis (FALS). We tested this hypothesis by ablating the gene encoding the copper chaperone for SOD1 (CCS) in a series of FALS-linked SOD1 mutant mice. Metabolic 64Cu labeling in SOD1-mutant mice lacking the CCS showed that the incorporation of copper...

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Source

Identifiers

PMID: 11889469
DOI: 10.1038/nn823

Genes

SOD1
CCS

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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