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Mitochondrially encoded transfer RNA alanine (MT-TA) is a short non-coding RNA, 69 nucleotides in length, encoded by the mitochondrial genome in humans[2][3]. Its primary function is to transport the amino acid alanine to mitochondrial ribosomes during mitochondrial translation, facilitating the synthesis of proteins essential for the oxidative phosphorylation (OXPHOS) system[1][2]. MT-TA operates as an adapter molecule, decoding mRNA codons into amino acids via its anticodon and is aminoacylated by a specific mitochondrial aminoacyl-tRNA synthetase[4][6]. Mutations in MT-TA have been implicated in various mitochondrial diseases, including myotonic dystrophy-like myopathy, by disrupting mitochondrial protein synthesis and, consequently, cellular energy production[1][2][7]. These mutations can manifest in tissues with high energy requirements, leading to diverse clinical presentations ranging from muscular dystrophy to generalized mitochondrial disorders[1][2].
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