AOX delays the onset of the lethal phenotype in a mouse model of Uqcrh (complex III) disease.

doi:10.1016/j.bbadis.2023.166760

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Article

AOX delays the onset of the lethal phenotype in a mouse model of Uqcrh (complex III) disease.

Biochimica et biophysica acta. Molecular basis of disease - 1 Oct 2023

Jacobs Howard T, Szibor Marten, Rathkolb Birgit, da Silva-Buttkus Patricia, Aguilar-Pimentel Juan Antonio, Amarie Oana V, Becker Lore, Calzada-Wack Julia, Dragano Nathalia, Garrett Lillian, Gerlini Raffaele, Hölter Sabine M, Klein-Rodewald Tanja, Kraiger Markus, Leuchtenberger Stefanie, Marschall Susan, Östereicher Manuela A, Pfannes Kristina, Sanz-Moreno Adrián, Seisenberger Claudia, Spielmann Nadine, Stoeger Claudia, Wurst Wolfgang, Fuchs Helmut, Hrabě de Angelis Martin, Gailus-Durner Valérie

Abstract excerpt

The alternative oxidase, AOX, provides a by-pass of the cytochrome segment of the mitochondrial respiratory chain when the chain is unavailable. AOX is absent from mammals, but AOX from Ciona intestinalis is benign when expressed in mice. Although non-protonmotive, so does not contribute directly to ATP production, it has been shown to modify and in some cases rescue phenotypes of respiratory-chain disease...

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PMID: 37230398
DOI: 10.1016/j.bbadis.2023.166760

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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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