Reactome: A Curated Pathway Database

MTHFD2, D2L oxidise 5,10-methylene-THF to 5,10-methenyl-THF

Stable Identifier
Homo sapiens
Locations in the PathwayBrowser

Mammalian mitochondria are able to produce formate from one-carbon (1C) donors such as serine, glycine and sarcosine. In mitochondria, the 1C units are oxidised to formate and transported to the cytosol, where the formate is reattached to tetrahydrofolate (THF) for use in de novo purine biosynthesis. The mitochondrial bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolases D2 and D2L (MTHFD2, MTHFD2L) mediate reversible reactions to convert 5,10-methylene-THF, via 5,10-methenyl-THF, to 10-formyl-THF. These enzymes have filled in missing gaps which existed in the transformation of 5,10-methylene-THF to formate in human mitochondria (Yang & MacKenzie 1993, Bolusani et al. 2011, Shin et al. 2014).

Literature References
Participant Of
This entity is regulated by:
Title Physical Entity Activity
methenyltetrahydrofolate cyclohydrolase activity of MTHFD2, D2L [mitochondrial matrix] MTHFD2, D2L [mitochondrial matrix] methenyltetrahydrofolate cyclohydrolase activity (0004477)
Orthologous Events