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Thus, they maintain electron flow under conditions when the classical respiratory chain is impaired, limiting excess production of oxygen radicals and supporting redox and metabolic homeostasis. AOX from Ciona intestinalis has been used to study and mitigate mitochondrial impairments in mammalian cell-lines, Drosophila disease models and, most recently, in the mouse, where multiple, lentivector-AOX transgenes conferred substantial expression in specific tissues. Here we describe a genetically tractable mouse model in which Ciona AOX has been targeted to the Rosa26 locus for ubiquitous expression. The AOXRosa26 mouse exhibited only subtle phenotypic effects on respiratory complex formation, oxygen consumption or the global metabolome, and showed an essentially normal physiology. AOX conferred robust resistance to inhibitors of the respiratory chain in organello, whilst animals exposed to a systemically applied LD50 dose of cyanide did not succumb. The AOXRosa26 mouse is a useful tool to investigate respiratory control mechanisms and to decipher mitochondrial disease aetiology in vivo.<\/jats:p>","DOI":"10.1242\/dmm.027839","type":"journal-article","created":{"date-parts":[[2017,1,9]],"date-time":"2017-01-09T16:05:08Z","timestamp":1483977908000},"source":"Crossref","is-referenced-by-count":26,"title":["Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology"],"prefix":"10.1242","author":[{"given":"Marten","family":"Szibor","sequence":"first","affiliation":[{"name":"Institute of Biotechnology, FI-00014 University of Helsinki, Finland"},{"name":"BioMediTech and Tampere University Hospital, FI-33014 University of Tampere, Finland"},{"name":"Max Planck Institute for Heart and Lung Research, D-61231 Bad Nauheim, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Praveen K.","family":"Dhandapani","sequence":"additional","affiliation":[{"name":"Institute of Biotechnology, 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