Entrez Summary: This gene encodes a GTPase that triggers back-translocation of the elongating ribosome during mitochondrial protein synthesis. The protein contains a highly conserved C-terminal domain not found in other GTPases that facilitates tRNA binding. The encoded protein is thought to prevent misincorporation of amino acids in stressful, suboptimal conditions. An allelic variant in this gene has been associated with early infantile epileptic encephalopathy-40. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2016].
UniProt Summary: Promotes mitochondrial protein synthesis. May act as a fidelity factor of the translation reaction, by catalyzing a one- codon backward translocation of tRNAs on improperly translocated ribosomes. Binds to mitochondrial ribosomes in a GTP-dependent manner. {ECO:0000255|HAMAP-Rule:MF_03137}.
Pfam DomainsGO Terms
Pfam Domains
EFG C
GTP EFTU
LepA C
GTP EFTU D2
Ras
GO Terms
ribosome binding
positive regulation of translation
positive regulation of cellular amide metabolic process
GTPase activity
regulation of translation
mitochondrial matrix
translation
GTP binding
mitochondrial inner membrane
regulation of cellular amide metabolic process
peptide biosynthetic process
posttranscriptional regulation of gene expression
amide biosynthetic process
peptide metabolic process
cellular amide metabolic process
mitochondrion
organonitrogen compound biosynthetic process
positive regulation of cellular protein metabolic process
cellular nitrogen compound biosynthetic process
positive regulation of protein metabolic process
cellular macromolecule biosynthetic process
macromolecule biosynthetic process
positive regulation of macromolecule biosynthetic process
positive regulation of cellular biosynthetic process
positive regulation of gene expression
gene expression
positive regulation of biosynthetic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)