| Function | Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis. Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism: thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA. Also required for mitochondrial translation by producing 5,10-methylenetetrahydrofolate.5,10-methylenetetrahydrofolate providing methyl donors to produce the taurinomethyluridine base at the wobble position of some mitochondrial tRNAs. Associates with mitochondrial DNA. In addition to its role in mitochondria, also plays a role in the deubiquitination of target proteins as component of the BRISC complex: required for IFNAR1 deubiquitination by the BRISC complex. |
| Protein Name | Serine Hydroxymethyltransferase - MitochondrialShmtGlycine HydroxymethyltransferaseSerine Methylase |
| Database Links | Reactome: R-HSA-196757Reactome: R-HSA-9013408Reactome: R-HSA-9837999 |
| Cellular Localisation | MitochondrionMitochondrion MatrixMitochondrion NucleoidMitochondrion Inner MembraneCytoplasmNucleusMainly Localizes In The MitochondrionAlso Found In The Cytoplasm And Nucleus As Part Of The Brisc Complex |
| Alternative Protein Names | Serine Hydroxymethyltransferase - Mitochondrial proteinShmt proteinGlycine Hydroxymethyltransferase proteinSerine Methylase proteinSHMT2 protein |
Information sourced from Uniprot.org
