Recombinant Human Elongation factor Tu, mitochondrial (TUFM)

Function

GTP hydrolase that promotes the GTP-dependent binding of aminoacyl-tRNA to the A-site of ribosomes during protein biosynthesis. Participates in mitochondrial translation (By similarity). Also plays a role in the regulation of autophagy and innate immunity (PubMed:22749352, PubMed:28407488). Recruits ATG5-ATG12 and NLRX1 at mitochondria and serves as a checkpoint of the RIGI-MAVS pathway (PubMed:28407488). In turn, inhibits RLR-mediated type I interferon while promoting autophagy (PubMed:22749352)

Subcellular Location

Mitochondrion matrix

Disease Association

Combined oxidative phosphorylation deficiency 4 (COXPD4) : A mitochondrial disease resulting in neonatal lactic acidosis, rapidly progressive encephalopathy, severely decreased mitochondrial protein synthesis, and combined deficiency of mtDNA-related mitochondrial respiratory chain complexes. [The disease is caused by variants affecting the gene represented in this entry]

Subunit

(Microbial infection) Interacts with Hantaan hantavirus glycoprotein N; this interaction contributes to the virus-induced degradation of mitochondria by autophagy, which leads to degradation of MAVS and inhibition of type I interferon (IFN) responses

Pathway

Protein biosynthesis; polypeptide chain elongation

• Wells J. et al. A mitochondrial elongation factor-like protein is over-expressed in tumours and differentially expressed in normal tissues. — FEBS Lett. (1995)
• Martin J. et al. The sequence and analysis of duplication-rich human chromosome 16. — Nature (2004)
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). — Genome Res. (2004)
• Woriax V.L. et al. Cloning, sequence analysis and expression of mammalian mitochondrial protein synthesis elongation factor Tu. — Biochim. Biophys. Acta (1995)
• Ling M. et al. The human mitochondrial elongation factor Tu (EF-Tu) gene: cDNA sequence, genomic localization, genomic structure, and identification of a pseudogene. — Gene (1997)
• Dunn M.J. et al. PubMed: CI-BLOECO93DHFFJ (MAR-)
• Gevaert K. et al. Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides. — Nat. Biotechnol. (2003)
• Lubec G. et al. PubMed: CI-2PBF0JF5JFFMO (MAR-)