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Article

Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS.

Human molecular genetics - 15 Jun 2008

Proescher Jody B, Son Marjatta, Elliott Jeffrey L, Culotta Valeria C

Abstract excerpt

The CCS copper chaperone is critical for maturation of Cu, Zn-superoxide dismutase (SOD1) through insertion of the copper co-factor and oxidization of an intra-subunit disulfide. The disulfide helps stabilize the SOD1 polypeptide, which can be particularly important in cases of amyotrophic latera...

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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: 18337307
DOI: 10.1093/hmg/ddn063
PMCID: PMC2900889

Genes

SOD1
CCS

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-18337307-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

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Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS.PMID 18337307Read ↗

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