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

Stable Identifier
Reaction [transition]
Homo sapiens
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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
Event Information
Catalyst Activity

formate-tetrahydrofolate ligase activity of MTHFD1L dimer [mitochondrial matrix]

Orthologous Events
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