Serine hydroxymethyltransferase, mitochondrial (SHMT2)

Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis (PubMed:24075985, PubMed:29364879, PubMed:25619277). Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of a single carbon from serine to tetrahydrofolate (PubMed:25619277).

Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism: thymidylate biosynthesis is needed to stop uracil accumulation in mtDNA (PubMed:21876188). Also required for mitochondrial translation by producing 5,10- methylenetetrahydrofolate; 5,10-methylenetetrahydrofolate providing methyl donors to produce the taurinomethyluridine base in the wobble position of some mitochondrial tRNAs (PubMed:29452640, PubMed:29364879).