Mitochondrially encoded tRNA leucine 2 (CUN) (MT-TL2)

Mitochondrially encoded tRNA leucine 2 (CUN) (MT-TL2, also known as TRNL2) is a mitochondrial gene encoding a transfer RNA responsible for recognizing the CUN codons during mitochondrial protein synthesis[1][3][4]. It spans 75 base pairs and is transcribed from the mitochondrial genome. MT-TL2 is critical for mitochondrial translation and thus for cellular energy production by supporting translation of 13 essential subunits of the oxidative phosphorylation system. Pathogenic mutations in MT-TL2 are associated with a spectrum of mitochondrial disorders, including progressive external ophthalmoplegia, mitochondrial encephalomyopathy, and may be risk variants for complex disorders such as polycystic ovary syndrome, cardiovascular diseases, and various cancers[1][2][3][4]. Variants in this gene can disrupt mitochondrial tRNA structure and function, leading to defective mitochondrial protein synthesis, impaired ATP generation, and increased reactive oxygen species[5]. MT-TL2 is classified as a non-coding RNA and is not considered a conventional druggable target such as a receptor, enzyme, or transporter[3][4][5].

Other names

TRNL2mitochondrially encoded tRNA-Leu (CUN) 2tRNA leucine 2 (CUN)MTTL2