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Article

In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 2 Nov 2011

Igoudjil Anissa, Magrané Jordi, Fischer Lindsey R, Kim Hyun Jeong, Hervias Isabel, Dumont Magali, Cortez Czrina, Glass Jonathan D, Starkov Anatoly A, Manfredi Giovanni

Abstract excerpt

Mutations in Cu,Zn superoxide dismutase (SOD1) are associated with familial amyotrophic lateral sclerosis (ALS). Mutant SOD1 causes a complex array of pathological events, through toxic gain of function mechanisms, leading to selective motor neuron degeneration. Mitochondrial dysfunction is among the well established toxic effects of mutant SOD1, but its mechanisms are just starting to be elucidated. A portion of...

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Identifiers

PMID: 22049426
DOI: 10.1523/JNEUROSCI.1965-11.2011
PMCID: PMC3249412

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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