Mitochondrially encoded transfer RNA for tryptophan (MT-TW)

Mitochondrially encoded tRNA-Trp is a small, non-coding RNA molecule transcribed from the mitochondrial genome that functions as the transfer RNA for tryptophan during mitochondrial protein synthesis[3]. This tRNA recognizes and decodes the UGA codon as tryptophan in the mitochondrial genetic code—a deviation from the nuclear code—enabling translation of key subunits of respiratory chain complexes[1]. Post-transcriptional modifications such as the addition of 5-taurinomethyluridine at the wobble position are essential for its function and stability[1][2]. Mutations in MT-TW may lead to defective mitochondrial translation, combined oxidative phosphorylation deficiencies, and are implicated in mitochondrial myopathies and multisystemic mitochondrial disorders[2][4]. While MT-TW is not considered a conventional therapeutic target like receptors or enzymes, detection of pathogenic mutations serves as a clinically relevant biomarker for mitochondrial disease diagnosis and monitoring[2][4].

Other names

MT-TWtrnWmitochondrial tRNA-Trpmitochondrial transfer RNA for tryptophan