Mitochondrially encoded tRNA valine (MT-TV)

Mitochondrially encoded tRNA valine (MT-TV, also called trnV or mitochondrial tRNA^Val) is a small transfer RNA found uniquely in mitochondria, where it is encoded by the mitochondrial genome at position 1602–1670 and spans 69 nucleotides in humans[1][2]. Its principal function is to deliver valine to the growing mitochondrial polypeptide chain during translation. In vertebrates, this molecule also plays a unique structural role by substituting for the missing 5S ribosomal RNA in the large subunit of the mitochondrial ribosome, facilitating proper mitochondrial protein synthesis[1][4]. MT-TV mutations are linked to several rare mitochondrial diseases, notably MELAS and Leigh's syndrome, as well as ataxia, cardiomyopathy, and other multi-system disorders, primarily through disruption of protein synthesis and energy metabolism[2][5]. No known drugs target this RNA directly, as it is not a conventional therapeutic target like receptors or enzymes.

Other names

trnVtRNA-Valmitochondrial tRNA^ValMTTV