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Article

Metabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex III deficiency due to a BCS1L mutation.

PloS one - 1 Jan 2012

Kotarsky Heike, Keller Matthias, Davoudi Mina, Levéen Per, Karikoski Riitta, Enot David P, Fellman Vineta

Abstract excerpt

BACKGROUND & AIMS: Liver is a target organ in many mitochondrial disorders, especially if the complex III assembly factor BCS1L is mutated. To reveal disease mechanism due to such mutations, we have produced a transgenic mouse model with c.232A>G mutation in Bcs1l, the causative mutation for GRAC...

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Identifiers

PMID: 22829922
DOI: 10.1371/journal.pone.0041156
PMCID: PMC3400604

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-22829922-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

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