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Letter

Nature Genetics 41, 833–837 (1 July 2009) | doi:10.1038/ng.390

Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome

Woranontee Weraarpachai , Hana Antonicka , Florin Sasarman , J|[uuml]|rgen Seeger , Bertold Schrank , Jill E Kolesar , Hanns Lochm|[uuml]|ller , Mario Chevrette , Brett A Kaufman , Rita Horvath & Eric A Shoubridge

Defects in mitochondrial translation are among the most common causes of mitochondrial disease, but the mechanisms that regulate mitochondrial translation remain largely unknown. In the yeast Saccharomyces cerevisiae, all mitochondrial mRNAs require specific translational activators, which recognize sequences in 5|[prime]| UTRs and mediate translation.