Mitochondrial transfer RNA Alanine (MT-TA)

Mitochondrial transfer RNA Alanine (MT-TA) is a non-coding RNA molecule encoded by the mitochondrial genome (mtDNA) that is essential for the translation of the 13 polypeptides forming the core of the oxidative phosphorylation (OXPHOS) system (NCBI Gene, 2024). It functions by delivering the amino acid alanine to mitochondrial ribosomes during protein synthesis. Mutations in the MT-TA gene, such as the m.5591G>A and m.5650G>A transitions, are clinically linked to mitochondrial diseases including chronic progressive external ophthalmoplegia (CPEO) and mitochondrial myopathy (MITOMAP, 2023). These mutations typically impair the structural stability or the aminoacylation efficiency of the tRNA, leading to a deficiency in mitochondrial-encoded proteins and subsequent cellular energy failure. Although no small-molecule drugs currently target MT-TA directly, it is a significant target for experimental genetic interventions, such as tRNA replacement and mitochondrial base editing, which aim to restore respiratory chain function (Finsterer et al., 2018; Gammage et al., 2018). The target is central to mitochondrial health, and its dysfunction is a hallmark of specific metabolic and neuromuscular disorders. Therapeutic strategies currently under investigation focus on bypassing the defective tRNA or correcting the underlying genetic mutation to restore ATP production.

Other names

MT-TATRNAAtRNA-AlaMitochondrial alanine tRNAtRNA Ala(UGC)

Restoration of mitochondrial translation through tRNA supplementation or gene correction.