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Fernandez-Ayala, Daniel J.M.,Sanz, Alberto,Vartiainen, Suvi,Kemppainen, Kia K.,Babusiak, Marek,Mustalahti, Eero,Costa, Rodolfo,Tuomela, Tea,Zeviani, Massimo,Chung, Jongkyeong,O'Dell, Kevin M.C.,Rustin Elsevier 2009 Cell metabolism Vol.9 No.5
<P><B>Summary</B></P><P>Defects in mitochondrial OXPHOS are associated with diverse and mostly intractable human disorders. The single-subunit alternative oxidase (AOX) found in many eukaryotes, but not in arthropods or vertebrates, offers a potential bypass of the OXPHOS cytochrome chain under conditions of pathological OXPHOS inhibition. We have engineered <I>Ciona intestinalis</I> AOX for conditional expression in <I>Drosophila melanogaster.</I> Ubiquitous AOX expression produced no detrimental phenotype in wild-type flies. However, mitochondrial suspensions from AOX-expressing flies exhibited a significant cyanide-resistant substrate oxidation, and the flies were partially resistant to both cyanide and antimycin. AOX expression was able to complement the semilethality of partial knockdown of both <I>cyclope</I> (COXVIc) and the complex IV assembly factor <I>Surf1</I>. It also rescued the locomotor defect and excess mitochondrial ROS production of flies mutated in <I>dj-1</I>β, a <I>Drosophila</I> homolog of the human Parkinson's disease gene <I>DJ1</I>. AOX appears to offer promise as a wide-spectrum therapeutic tool in OXPHOS disorders.</P>