Mutations in MTFMT underlie a human disorder of formylation causing impaired mitochondrial translation

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Mutations in MTFMT underlie a human disorder of formylation causing impaired mitochondrial translation.

The metazoan mitochondrial translation machinery is unusual in having a single tRNA(Met) that fulfills the dual role of the initiator and elongator tRNA(Met). A portion of the Met-tRNA(Met) pool is formylated by mitochondrial methionyl-tRNA formyltransferase (MTFMT) to generate N-formylmethionine-tRNA(Met) (fMet-tRNA(met)), which is used for translation initiation; however, the requirement of f...

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ژورنال

عنوان ژورنال: Mitochondrion

سال: 2012

ISSN: 1567-7249

DOI: 10.1016/j.mito.2012.07.084