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Article

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities.

Human molecular genetics - 1 Dec 2009

Magrané Jordi, Hervias Isabel, Henning Matthew S, Damiano Maria, Kawamata Hibiki, Manfredi Giovanni

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by motor neuron degeneration. Mutations in Cu,Zn-superoxide dismutase (SOD1) are responsible for 20% of familial ALS cases via a toxic gain of function. In mutant SOD1 transgenic mice, mitochondria of spinal motor neurons develop abnormal morphology, bioenergetic defects and degeneration, which are presumably implicated in disease...

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Source

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Identifiers

PMID: 19779023
DOI: 10.1093/hmg/ddp421
PMCID: PMC2773270

Genes

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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