Recombinant Human Elongation factor Tu, mitochondrial (TUFM)

Uniprot Id

P49411

Target Species

Human

Target Name

TUFM

Target Full Name

Elongation factor Tu, mitochondrial

Target Function

Promotes the GTP-dependent binding of aminoacyl-tRNA to the A-site of ribosomes during protein biosynthesis. Plays also a role in the regulation of autophagy and innate immunity. Recruits ATG5-ATG12 and NLRX1 at mitochondria and serves as a checkpoint of the RIG-I/DDX58-MAVS pathway. In turn, inhibits RLR-mediated type I interferon while promoting autophagy.

Target Involvement

Combined oxidative phosphorylation deficiency 4 (COXPD4)

Target Subcellular Location

Mitochondrion.

Target Protein Families

TRAFAC class translation factor GTPase superfamily, Classic translation factor GTPase family, EF-Tu/EF-1A subfamily

Target Research Area

Metabolism, Epigenetics and Nuclear Signaling

Target Synonyms

COXPD4; EF Tu; EF TuMT; EF-Tu; EF-TuMT; EFTu; EFTU_HUMAN; EFTuMT; Elongation factor Tu; Elongation factor Tu mitochondrial; mitochondrial; P43; Tu translation elongation factor; mitochondrial; Tufm

Target Background

This gene encodes a protein which participates in protein translation in mitochondria. Mutations in this gene have been associated with combined oxidative phosphorylation deficiency resulting in lactic acidosis and fatal encephalopathy. A pseudogene has been identified on chromosome 17.