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Article

A molecular chaperone activity of CCS restores the maturation of SOD1 fALS mutants.

Scientific reports - 12 Dec 2017

Luchinat Enrico, Barbieri Letizia, Banci Lucia

Abstract excerpt

Superoxide dismutase 1 (SOD1) is an important metalloprotein for cellular oxidative stress defence, that is mutated in familiar variants of Amyotrophic Lateral Sclerosis (fALS). Some mutations destabilize the apo protein, leading to the formation of misfolded, toxic species. The Copper Chaperone for SOD1 (CCS) transiently interacts with SOD1 and promotes its correct maturation by transferring copper and...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 29234142
DOI: 10.1038/s41598-017-17815-y
PMCID: PMC5727297

Genes

CCS
SOD1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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