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