GFM2

Mitochondrial GTPase that mediates the disassembly of ribosomes from messenger RNA at the termination of mitochondrial protein biosynthesis (PubMed:19716793, PubMed:33878294). Acts in collaboration with MRRF (PubMed:19716793, PubMed:33878294). Promotes mitochondrial ribosome recycling by dissolution of intersubunit contacts (PubMed:33878294). GTP hydrolysis follows the ribosome disassembly and probably occurs on the ribosome large subunit (PubMed:19716793). Not involved in the GTP-dependent ribosomal translocation step during translation elongation (PubMed:19716793).

Combined oxidative phosphorylation deficiency 39

COXPD39

An autosomal recessive disorder due to mitochondrial dysfunction and characterized by global developmental delay, axial hypotonia, dystonia, dysarthria, impaired intellectual development with poor speech, and deficiencies of the mitochondrial respiratory chain enzyme complexes. Neuroimaging shows abnormalities in the putamen and caudate nuclei, along with subcortical white matter involvement.

None

The disease is caused by variants affecting the gene represented in this entry.

Belongs to the TRAFAC class translation factor GTPase superfamily. Classic translation factor GTPase family. EF-G/EF-2 subfamily.

Widely expressed.

• Mitochondrion

EFG2, MSTP027, GFM2, RRF2mt, Elongation factor G2, EF-G2mt, mEF-G 2, hEFG2

• entrezGene:84340
• omim:606544
• swissprot:Q969S9

Proteins

86601Da