GFM1 Antibody – Rabbit Polyclonal

EFG1: 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. Defects in GFM1 are the cause of combined oxidative phosphorylation deficiency type 1 (COXPD1). It leads to early fatal progressive hepatoencephalopathy. Belongs to the GTP-binding elongation factor family. EF-G/EF-2 subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Translation elongation; RNA-binding

Chromosomal Location of Human Ortholog: 3q25

Cellular Component: mitochondrial matrix

Molecular Function: GTPase activity; protein binding; GTP binding; translation elongation factor activity

Biological Process: mitochondrial translation; organelle organization and biogenesis

Disease: Combined Oxidative Phosphorylation Deficiency 1