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Article

Dimerization, oligomerization, and aggregation of human amyotrophic lateral sclerosis copper/zinc superoxide dismutase 1 protein mutant forms in live cells.

The Journal of biological chemistry - 23 May 2014

Kim Jiho, Lee Honggun, Lee Joo Hyun, Kwon Do-yoon, Genovesio Auguste, Fenistein Denis, Ogier Arnaud, Brondani Vincent, Grailhe Regis

Abstract excerpt

More than 100 copper/zinc superoxide dismutase 1 (SOD1) genetic mutations have been characterized. These mutations lead to the death of motor neurons in ALS. In its native form, the SOD1 protein is expressed as a homodimer in the cytosol. In vitro studies have shown that SOD1 mutations impair the dimerization kinetics of the protein, and in vivo studies have shown that SOD1 forms aggregates in patients with...

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Identifiers

PMID: 24692554
DOI: 10.1074/jbc.M113.542613
PMCID: PMC4031559

Genes

SOD1

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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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