GTP-dependent ribosome recycling factor mitochondrial 2 (GFM2)

GTP-dependent ribosome recycling factor mitochondrial 2 (GFM2) is a mitochondrial-localized GTPase essential for the termination phase of mitochondrial protein synthesis. It promotes the disassembly and recycling of the mitochondrial ribosome after translation termination by hydrolyzing GTP, enabling successive rounds of protein biosynthesis. GFM2 is not involved in the elongation step, distinguishing it from its paralog GFM1. Mutations in GFM2 disrupt mitochondrial protein synthesis, resulting in defects in oxidative phosphorylation (OXPHOS) complexes, leading to combined oxidative phosphorylation deficiency, Leigh syndrome, neurodevelopmental delay, and other mitochondrial diseases. The protein shows homology to bacterial elongation factor G but is functionally distinct in humans. GFM2 mutations have been identified in autosomal-recessive syndromes with varied presentation, with mutations often resulting in loss-of-function alleles subject to nonsense-mediated decay. There are no known pharmacological inhibitors or approved drugs specifically targeting GFM2, and it is primarily of interest for the diagnosis and understanding of mitochondrial diseases

Other names

Ribosome-releasing factor 2, mitochondrialEFG2EF-G2mtMRRF2MSTP027RRF2mtmEF-G 2hEFG2FLJ21661Elongation factor G 2, mitochondrialElongation factor G2ribosome releasing factor 2mitochondrial ribosome-releasing factor 2G elongation factor mitochondrial 2mitochondrial elongation factor G2MST027RRFRRF2ribosome-releasing factor 2, mitochondrial