Mitochondrial translation release factor in rescue (MTRFR)

Mitochondrial translation release factor in rescue (MTRFR) is a mitochondrial matrix protein encoded by a nuclear gene and is involved in peptide chain termination within the mitochondrial translation system[1][3][6]. It participates in mitoribosome-associated quality control: together with its partner MTRES1, it acts to resolve translation stalls by ejecting unfinished peptide chains and peptidyl-tRNA from stalled ribosomes, thereby preventing aberrant protein synthesis within mitochondria[1][2][6]. Mutations in MTRFR are linked to severe mitochondrial diseases, including spastic paraplegia 55 and combined oxidative phosphorylation deficiency 7, which feature decreased mitochondrial translation and impaired cellular energy production[1][3].

Other names

C12orf65My030FLJ38663SPG55COXPD7mtRF-Rprobable peptide chain release factor C12orf65, mitochondrial