MTHFD1L transforms 10-formyl-THF to HCOOH and THF

Stable Identifier
R-HSA-6801456
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
Summation

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
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
Participants
Participant Of
Event Information
Catalyst Activity
Catalyst Activity
Title
formate-tetrahydrofolate ligase activity of MTHFD1L dimer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored
Reviewed