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Article

Oligomerization of mutant SOD1 in mitochondria of motoneuronal cells drives mitochondrial damage and cell toxicity.

Antioxidants & redox signaling - 1 Jul 2009

Cozzolino Mauro, Pesaresi Maria Grazia, Amori Ilaria, Crosio Claudia, Ferri Alberto, Nencini Monica, Carrì Maria Teresa

Abstract excerpt

Increasing evidence indicates that the accumulation and aggregation of mutant Cu,Zn superoxide dismutase (mutSOD1) in spinal cord mitochondria is implicated in the pathogenesis of familial amyotrophic lateral sclerosis (FALS). Although the mechanisms underlying this effect are only partially understood, a deficit in the import mechanism of mutSOD1 and/or in its folding and maturation inside mitochondria is likely...

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Source

Identifiers

PMID: 19344252
DOI: 10.1089/ars.2009.2545

Genes

CCS
SOD1

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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