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Article

Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy.

Cell metabolism - 1 Apr 2008

Kruse Shane E, Watt William C, Marcinek David J, Kapur Raj P, Schenkman Kenneth A, Palmiter Richard D

Abstract excerpt

To study effects of mitochondrial complex I (CI, NADH:ubiquinone oxidoreductase) deficiency, we inactivated the Ndufs4 gene, which encodes an 18 kDa subunit of the 45-protein CI complex. Although small, Ndufs4 knockout (KO) mice appeared healthy until approximately 5 weeks of age, when ataxic signs began, progressing to death at approximately 7 weeks. KO mice manifested encephalomyopathy including a retarded...

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Source

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Identifiers

PMID: 18396137
DOI: 10.1016/j.cmet.2008.02.004
PMCID: PMC2593686

Genes

NDUFS4

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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