Reactome | MTHFD1L ligates HCOOH to THF to form 10-formyl-THF

MTHFD1L ligates HCOOH to THF to form 10-formyl-THF

Stable Identifier

R-HSA-5696839

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

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MTHFD1L ligates HCOOH to THF to form 10-formyl-THF

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All C1-tetrahydrofolate (C1-THF) synthases characterised to date are trifunctional, containing the activities of 5,10-methylene-THF dehydrogenase, 5,10-methenyl-THF cyclohydrolase, and 10-formyl-THF synthetase. Mitochondrial monofunctional C1-tetrahydrofolate synthase (MTHFD1L) only possesses 10-formyl-THF synthetase activity and it reversibly ligates formate (HCOOH) to tetrahydrofolate (THF), forming 10-formyltetrahydrofolate (10-formyl-THF) (Prasannan et al. 2003, Walkup & Appling 2005). MTHFD1L is functional as a homodimer and could be a missing reaction in one-carbon folate metabolism linking the metabolism of formate from the cytosol to mitochondria (Pike et al. 2010).

Literature References

PubMed ID	Title	Journal	Year
16171773	Enzymatic characterization of human mitochondrial C1-tetrahydrofolate synthase Walkup, AS, Appling, DR	Arch. Biochem. Biophys.	2005
19948730	Mitochondrial C1-tetrahydrofolate synthase (MTHFD1L) supports the flow of mitochondrial one-carbon units into the methyl cycle in embryos Pike, ST, Rajendra, R, Artzt, K, Appling, DR	J. Biol. Chem.	2010
12937168	Human mitochondrial C1-tetrahydrofolate synthase: gene structure, tissue distribution of the mRNA, and immunolocalization in Chinese hamster ovary calls Prasannan, P, Pike, S, Peng, K, Shane, B, Appling, DR	J. Biol. Chem.	2003