Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
10-formyltetrahydrofolate + L-methionyl-tRNAfMet | Homo sapiens | - |
tetrahydrofolate + N-formylmethionyl-tRNAfMet | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
10-formyltetrahydrofolate + L-methionyl-tRNAfMet | - |
Homo sapiens | tetrahydrofolate + N-formylmethionyl-tRNAfMet | - |
? |
Synonyms | Comment | Organism |
---|---|---|
mitochondrial methionyl-tRNA formyltransferase | - |
Homo sapiens |
MTFMT | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | heterozygous mutations in MTFMT occur in two unrelated children presenting with Leigh syndrome and combined OXPHOS deficiency. The patient fibroblasts have dramatically reduced fMet-tRNAMet levels and an abnormal formylation profile of mitochondrially translated COX1, and patient fibroblasts exhibit severe defects in mitochondrial translation that can be rescued by exogenous expression of MTFMT. Mitochondrial tRNAMet pools are abnormal in patient fibroblasts, phenotypes, overview | Homo sapiens |
physiological function | MTFMT is critical for efficient human mitochondrial translation | Homo sapiens |