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GFM1

Function

Mitochondrial GTPase that catalyzes the GTP-dependent ribosomal translocation step during translation elongation. During this step, the ribosome changes from the pre-translocational (PRE) to the post-translocational (POST) state as the newly formed A-site-bound peptidyl-tRNA and P-site-bound deacylated tRNA move to the P and E sites, respectively. Catalyzes the coordinated movement of the two tRNA molecules, the mRNA and conformational changes in the ribosome. Does not mediate the disassembly of ribosomes from messenger RNA at the termination of mitochondrial protein biosynthesis.

Involvement in disease

Combined oxidative phosphorylation deficiency 1

COXPD1

A mitochondrial disease resulting in early rapidly progressive hepatoencephalopathy.

None

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

Pathway

Protein biosynthesis; polypeptide chain elongation.

Sequence Similarities

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

Cellular localization

Alternative names

EFG, EFG1, GFM, GFM1, EF-Gmt, Elongation factor G1, mEF-G 1, hEFG1

swissprot:Q96RP9 omim:606639 entrezGene:85476