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. Author manuscript; available in PMC: 2020 Jan 2.
Published in final edited form as: Birth Defects Res. 2019 Sep 13;111(19):1520–1534. doi: 10.1002/bdr2.1591

FIGURE 1.

FIGURE 1

Compartmentalization of folate-dependent one-carbon metabolism. In mammalian mitochondria, monofunctional MTHFD1L catalyzes the 10-formyl-THF (CHO-THF) synthetase reaction. This enzyme is missing in Mthfd1l knockout mice. Bifunctional MTHFD2 or MTHFD2L enzymes catalyse 5,10-methenyl-THF (CH+-THF) cyclohydrolase and 5,10-methylene-THF (CH2-THF) dehydrogenase activities. MTHFD1 is the cytoplasmic trifunctional C1-THF synthase that catalyzes 10-formyl-THF synthetase, 5,10-methenyl-THF cyclohydrolase, and 5,10-methylene-THF dehydrogenase activities. SHMT1 and SHMT2 represent cytoplasmic and mitochondrial serine hydroxymethyltransferase isozymes, respectively. Gray ovals represent putative metabolite transporters. CH3-THF, 5-methyl-THF; dTMP, thymidylate