Article
Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome.
Nature genetics - 1 Jul 2009
Weraarpachai Woranontee, Antonicka Hana, Sasarman Florin, Seeger Jürgen, Schrank Bertold, Kolesar Jill E, Lochmüller Hanns, Chevrette Mario, Kaufman Brett A, Horvath Rita, Shoubridge Eric A
Abstract excerpt
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' UTRs and mediate translation. As mammalian mitochondrial mRNAs do not have significant 5' UTRs,...
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