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Article

Mutant SOD1 from spinal cord of G93A rats is destabilized and binds to inner mitochondrial membrane.

Neurobiology of disease - 1 Dec 2008

Ahtoniemi Toni, Jaronen Merja, Keksa-Goldsteine Velta, Goldsteins Gundars, Koistinaho Jari

Abstract excerpt

Mutations in Cu/Zn superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS). Mechanisms of mutant SOD1 toxicity are unknown, but increased SOD1 activity can boost production of reactive oxygen species (ROS) in the mitochondrial intermembrane space (IMS). Using non-reducing SDS-PAGE we found that in G93A-SOD1 rats the mutant SOD1 was prominently destabilized only in the diseased spinal cord, where...

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Source

Identifiers

PMID: 18817872
DOI: 10.1016/j.nbd.2008.08.010

Genes

SOD1
SDS

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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