Mitochondrial translation release factor 1 (MTRF1)

Mitochondrial translation release factor 1 (MTRF1) is an essential component of the human mitochondrial translation machinery. Unlike the canonical release factor mtRF1a, which recognizes UAA and UAG stop codons, MTRF1 specifically mediates translation termination at the non-canonical mitochondrial stop codons AGA and AGG[1][2][4][5]. This function is critical for the proper synthesis of mitochondrially encoded proteins, such as COX1 and ND6. MTRF1 contains a unique codon-recognition domain that enables it to recognize these atypical stop codons through a complex network of interactions with the mRNA and ribosomal RNA, and possesses a highly conserved GGQ motif necessary for catalyzing the release of the nascent polypeptide from the mitochondrial ribosome[1][2][4][5]. Disruption of MTRF1 function leads to ribosomal stalling and impaired synthesis of crucial mitochondrial proteins, but no current drugs are known to target or modulate MTRF1 directly. The role of MTRF1 is vital in maintaining mitochondrial protein homeostasis, with potential indirect implications in mitochondrial disorders[1][2][4][5].

Other names

Peptide chain release factor 1, mitochondrialMRF-1MtRF-1RF1MTTRF1MGC47721mitochondrial peptide chain release factor 1mtRF-1

Hydrolysis of peptidyl-tRNA at mitochondrial ribosome when non-canonical stop codons AGA or AGG encountered, thus releasing the nascent polypeptide[1][2][4][5].