Mitochondrially encoded tRNA-Ser (UCN) 1 (MT-TS1)

Mitochondrially encoded tRNA-Ser (UCN) 1, abbreviated as MT-TS1, is a small mitochondrial transfer RNA molecule that enables the incorporation of the amino acid serine (coded by the UCN anticodon) into proteins during mitochondrial translation. It is critical for correct mitochondrial protein synthesis, particularly for proteins involved in oxidative phosphorylation, which is central to cellular energy production. Pathogenic mutations in MT-TS1 disrupt normal mitochondrial protein synthesis and cause diverse clinical syndromes, mainly affecting organs with high energy demand such as muscle (myopathy, exercise intolerance, myoclonic epilepsy) and sensory tissues (hearing loss, keratoderma)[1][2][6][8]. MT-TS1 itself is not a drug target, but genetic testing for MT-TS1 mutations is used to aid diagnosis in mitochondrial diseases[1][6][8].

Other names

MTTS1TRNS1tRNA serine 1 (UCN)tRNASer(UCN)

Not applicable. There are no drug mechanisms associated with this tRNA; disease processes result from genetic mutations affecting its function