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Mitochondrially encoded transfer RNA tyrosine (MT-TY, trnY) is a 69-nucleotide tRNA molecule encoded by the mitochondrial *MT-TY* gene. It functions within mitochondria to transport tyrosine during translation of mitochondrial-encoded proteins. Pathogenic variants in MT-TY disrupt mitochondrial protein synthesis, resulting in impaired oxidative phosphorylation and multisystem mitochondrial disease phenotypes, including progressive neurodegeneration, myopathy, external ophthalmoplegia, and hearing/vision loss[4]. MT-TY undergoes complex post-transcriptional modifications, which are required for normal folding and function. Biomarkers for related mitochondrial diseases include elevated lactate and GDF15. MT-TY is sometimes referenced in disease studies as a biomarker target, but is not considered a conventional drug target or receptor[4][5].
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