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Article

Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis.

The Journal of biological chemistry - 29 Aug 2008

Furukawa Yoshiaki, Kaneko Kumi, Yamanaka Koji, O'Halloran Thomas V, Nukina Nobuyuki

Abstract excerpt

Dominant mutations in Cu,Zn-superoxide dismutase (SOD1) cause a familial form of amyotrophic lateral sclerosis (fALS), and aggregation of mutant SOD1 has been proposed to play a role in neurodegeneration. A growing body of evidence suggests that fALS-causing mutations destabilize the native structure of SOD1, leading to aberrant protein interactions for aggregation. SOD1 becomes stabilized and enzymatically...

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Source

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Identifiers

PMID: 18552350
DOI: 10.1074/jbc.M802083200
PMCID: PMC3259764

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.

Related research

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Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis.PMID 18552350Read ↗

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Disulfide scrambling describes the oligomer formation of superoxide dismutase (SOD1) proteins in the familial form of amyotrophic lateral sclerosis.Semantic proximity Read ↗

The effects of glutaredoxin and copper activation pathways on the disulfide and stability of Cu,Zn superoxide dismutase.Semantic proximity Read ↗

Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space.Semantic proximity Read ↗

Molecular and pharmacological chaperones for SOD1.Semantic proximity Read ↗

Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation.Semantic proximity Read ↗

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Oligomerization of mutant SOD1 in mitochondria of motoneuronal cells drives mitochondrial damage and cell toxicity.Semantic proximity Read ↗

Mutations in Superoxide Dismutase 1 (Sod1) Linked to Familial Amyotrophic Lateral Sclerosis Can Disrupt High-Affinity Zinc-Binding Promoted by the Copper Chaperone for Sod1 (Ccs).Semantic proximity Read ↗

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