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Promotes mitochondrial protein synthesis.
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Homozygous variant in the GUF1 gene identified in the three siblings with West syndrome.
The structure provides insights into the tRNA-remodeling function of EF-4 on the ribosome and suggests that the displacement of the CCA (show FBN2 ELISA Kits)-end of the A-site tRNA away from the peptidyl transferase center (PTC (show F9 ELISA Kits)) is functionally significant.
Genetic ablation of mitochondrial EF4 (mtEF4) in mice causes testis-specific (show AIF1 ELISA Kits) dysfunction in oxidative phosphorylation, leading to male infertility.
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 (By similarity).
GTP-binding protein GUF1 homolog
, GTPase GUF1
, GTPase of unknown function 1
, elongation factor 4 homolog
, ribosomal back-translocase
, translation factor GUF1, mitochondrial
, GTPase Guf1
, translation factor Guf1, mitochondrial
, hypothetical protein