MTHFD1L transforms 10-formyl-THF to HCOOH and 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). Under most conditions, the majority of one-carbon units for cytoplasmic processes are derived from mitochondrial formate.

Literature References
PubMed ID Title Journal Year
19948730 Mitochondrial C1-tetrahydrofolate synthase (MTHFD1L) supports the flow of mitochondrial one-carbon units into the methyl cycle in embryos

Artzt, K, Pike, ST, Rajendra, R, Appling, DR

J. Biol. Chem. 2010
16171773 Enzymatic characterization of human mitochondrial C1-tetrahydrofolate synthase

Appling, DR, Walkup, AS

Arch. Biochem. Biophys. 2005
12937168 Human mitochondrial C1-tetrahydrofolate synthase: gene structure, tissue distribution of the mRNA, and immunolocalization in Chinese hamster ovary calls

Shane, B, Appling, DR, Peng, K, Prasannan, P, Pike, S

J. Biol. Chem. 2003
Catalyst Activity

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

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