Reaction: MTHFD1L dimer ligates HCOOH to THF to form 10-formyl-THF
- in pathway: Metabolism of folate and pterines
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).
Reaction - small molecule participants:
Pi [mitochondrial matrix]
ADP [mitochondrial matrix]
10-formyl-THF [mitochondrial matrix]
HCOOH [mitochondrial matrix]
THF [mitochondrial matrix]
ATP [mitochondrial matrix]
Reactome.org reaction link: R-HSA-5696839
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Reaction input - small molecules:
formate
(6S)-5,6,7,8-tetrahydrofolic acid
ATP(4-)
Reaction output - small molecules:
hydrogenphosphate
ADP(3-)
10-formyltetrahydrofolate(2-)
Reactome.org link: R-HSA-5696839