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Mitochondrially encoded transfer RNA tyrosine (MT-TY)

Target
MT-TY
Molecular classification
Transfer RNA (tRNA), small noncoding RNA, belongs to mitochondrial tRNA family
01

Overview

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].

Other names
MT-TYtrnYmt-tRNA^Tyr^mitochondrial tRNA-Tyr
02

Biological functions

Mitochondrial protein translation (specifically decoding tyrosine codons during synthesis of mitochondrial-encoded proteins)Involvement in oxidative phosphorylation (by enabling translation of subunits of mitochondrial respiratory chain complexes)
03

Disease associations

Mitochondrial disease (especially multisystem mitochondrial disorders, neurodegenerative disease, myopathy, ptosis, ophthalmoplegia, hearing and vision loss)
04

Biomarkers

Blood and CSF lactateGrowth differentiation factor 15 (GDF15)Muscle biopsy evidence (COX-negative fibers, ragged red fibers with high tRNA variant heteroplasmy)

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