TSFM

Synthesis of the 13 mitochondrial-encoded proteins occurs on a dedicated mitochondrial translation apparatus similar to that found in prokaryotes and requires, in addition to the tRNAs and rRNAs encoded in mtDNA, the concerted action of several translation factors and a large number of mitochondrial ribosomal proteins, all of which are encoded by nuclear genes. The TSFM gene encodes a mitochondrial translation elongation factor (Smeitink et al., 2006 [PubMed 17033963]).[supplied by OMIM

Full Name

Ts translation elongation factor, mitochondrial

Function

Associates with the EF-Tu.GDP complex and induces the exchange of GDP to GTP. It remains bound to the aminoacyl-tRNA.EF-Tu.GTP complex up to the GTP hydrolysis stage on the ribosome.

Biological Process

Biological Process mitochondrial translational elongation Source:GO_Central1 Publication
Biological Process regulation of DNA-templated transcription elongation Source:ProtInc1 Publication
Biological Process regulation of mitochondrial translation Source:UniProtKB1 Publication
Biological Process translational elongation Source:GO_Central1 Publication

Cellular Location

Mitochondrion

Involvement in disease

Combined oxidative phosphorylation deficiency 3 (COXPD3):
A mitochondrial disease resulting in severe metabolic acidosis with encephalomyopathy or with hypertrophic cardiomyopathy. Patients show a severe defect in mitochondrial translation leading to a failure to assemble adequate amounts of three of the oxidative phosphorylation complexes.