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Article

Mitochondrial respiration without ubiquinone biosynthesis.

Human molecular genetics - 1 Dec 2013

Wang Ying, Hekimi Siegfried

Abstract excerpt

Ubiquinone (UQ), a.k.a. coenzyme Q, is a redox-active lipid that participates in several cellular processes, in particular mitochondrial electron transport. Primary UQ deficiency is a rare but severely debilitating condition. Mclk1 (a.k.a. Coq7) encodes a conserved mitochondrial enzyme that is necessary for UQ biosynthesis. We engineered conditional Mclk1 knockout models to study pathogenic effects of UQ...

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Identifiers

PMID: 23847050
DOI: 10.1093/hmg/ddt330
PMCID: PMC3888124

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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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