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Mitochondrially encoded tRNA phenylalanine (MT-TF, trnF) is a transfer RNA transcribed from the mitochondrial genome, responsible for recognizing phenylalanine codons (UUU, UUC) during mitochondrial translation and delivering phenylalanine to the growing polypeptide chain[1][6]. Mutations in MT-TF disrupt aminoacylation, structural integrity, and translation factor binding[2][3]. These defects can cause a spectrum of mitochondrial diseases including MERRF syndrome, myopathies, and complex IV deficiencies, often manifesting as muscle weakness, neurological symptoms, and metabolic disorders[2][3][5]. Pathogenic variants in MT-TF are not directly targeted by drugs, but disease-linked mutations are biomarkers for diagnosis. MT-TF can also generate mt-tRNA-derived fragments (mt-tRFs) that may have roles in intercellular signaling and mitochondrial-nuclear communication[4]. Key insights: - MT-TF is a mitochondrial transfer RNA, not an enzyme, receptor, or transporter. - It is crucial for mitochondrial translation fidelity. - Pathogenic point mutations in MT-TF lead to severe mitochondrial diseases but are not directly modulated therapeutically[2][3][5].
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