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mitochondrial elongation factor G1; elongation factor G, mitochondrial; EF-Gmt; elongation factor G 1, mitochondrial; mEF-G 1; elongation factor G1; hEFG1 (GFM1, EFG, EFG1, GFM)

Function: - mitochondrial GTPase - catalyzes 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 & P-site bound deacylated tRNA move to the P-site & E-site, respectively - catalyzes the coordinated movement of the two tRNA molecules, the mRNA & conformational changes in the ribosome - does not mediate the disassembly of ribosomes from mRNA at the termination of mitochondrial protein biosynthesis - protein biosynthesis; polypeptide chain elongation Structure: - belongs to the GTP-binding elongation factor family EF-G/EF-2 subfamily Compartment: mitochondria (putative) Alternative splicing: named isoforms=2 Pathology: - defects in GFM1 are the cause of combined oxidative phosphorylation deficiency type 1y

Related

GFM1 gene mutation

General

mitochondrial protein translation elongation factor

Properties

SIZE: entity length = 751 aa MW = 83 kD COMPARTMENT: mitochondria MOTIF: GTP-binding site SITE: 53-60 GTP-binding site SITE: 120-124 GTP-binding site SITE: 174-177

Database Correlations

OMIM correlations MORBIDMAP 606639 UniProt Q96RP9 PFAM correlations Entrez Gene 85476 Kegg hsa:85476

References

  1. UniProt :accession Q96RP9
  2. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/GFM1