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Article

Cellular and animal models for mitochondrial complex I deficiency: a focus on the NDUFS4 subunit.

IUBMB life - 1 Mar 2013

Breuer Megan E, Willems Peter H G M, Smeitink Jan A M, Koopman Werner J H, Nooteboom Marco

Abstract excerpt

To allow the rational design of effective treatment strategies for human mitochondrial disorders, a proper understanding of their biochemical and pathophysiological aspects is required. The development and evaluation of these strategies require suitable model systems. In humans, inherited complex I (CI) deficiency is one of the most common deficiencies of the mitochondrial oxidative phosphorylation system. During...

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Source

Identifiers

PMID: 23378164
DOI: 10.1002/iub.1127

Genes

NDUFS4

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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