Mitochondrially encoded tRNA isoleucine (MT-TI)

Mitochondrially encoded tRNA isoleucine (MT-TI, also known as trnI or mt-tRNA^Ile) is a 69-nucleotide RNA molecule encoded by the mitochondrial genome. It functions as a transfer RNA (tRNA) specifically responsible for incorporating isoleucine into mitochondrial-encoded proteins during mitochondrial translation. The maturation of MT-TI involves a series of enzymatic steps, including 5′ and 3′ processing, methylation, and CCA addition, mediated by multi-protein complexes such as TRMT10C/SDR5C1 and TRNT1[1]. Mutations in the MT-TI gene are associated with defects in mitochondrial protein biosynthesis and are implicated in various mitochondrial diseases, primarily affecting energy-intensive tissues such as muscle and brain. Such mutations can cause altered tRNA structure, decreased aminoacylation, impaired translation, and reduced respiratory chain complex activities[2]. MT-TI is not a receptor, enzyme, or classic druggable protein target, but rather a non-coding structural RNA essential for mitochondrial function.

Other names

trnImt-tRNA^IletRNA-Ile^(UAU)mitochondrial tRNA-Ile

Not applicable (as a tRNA, it is not a classical drug target; its mutations may affect mitochondrial translation or respiratory chain function, but it is not a therapeutic target)