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Proteins/UNIPROT:Q9H903

MTHFD2L

Protein·UNIPROT:Q9H903Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase 2, mitochondrial

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Bifunctional mitochondrial folate-interconverting enzyme that has both NAD/NADP-dependent methylenetetrahydrofolate dehydrogenase and methenyltetrahydrofolate cyclohydrolase activities..

Function from UniProtKB (Q9H903), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

Statements

18 statements

18 statements · 5 reference databases · Subject: MTHFD2L · Sources: Human-GEM, Reactome, Rhea, UniProt, WikiPathways

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MTHFD2L participates in Disorders of folate metabolism and transportPathwayWikiPathways WP4259Inferred only
MTHFD2L participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
MTHFD2L participates in Metabolism of folate and pterinesPathwayReactome R-HSA-196757Inferred only
MTHFD2L participates in Metabolism of vitamins and cofactorsPathwayReactome R-HSA-196854Inferred only
MTHFD2L participates in Metabolism of water-soluble vitamins and cofactorsPathwayReactome R-HSA-196849Inferred only
MTHFD2L participates in Trans sulfuration one carbon metabolism and related pathwaysPathwayWikiPathways WP2525Inferred only
MTHFD2L requires the cofactor magnesium(2+)CompoundUniProt Q9H903Inferred only
(6R)-10-formyltetrahydrofolate is a substrate of MTHFD2LCompoundRhea RHEA:23700Inferred only
(6R)-5,10-methenyltetrahydrofolate is a substrate of MTHFD2LCompoundHuman-GEM MAR04440Inferred only
(6R)-5,10-methenyltetrahydrofolate is a substrate of MTHFD2LCompoundHuman-GEM MAR04505Inferred only
(6R)-5,10-methenyltetrahydrofolate is a substrate of MTHFD2LCompoundRhea RHEA:22892Inferred only
(6R)-5,10-methenyltetrahydrofolate is a substrate of MTHFD2LCompoundRhea RHEA:23700Inferred only

Show the remaining 6

(6R)-5,10-methylene-5,6,7,8-tetrahydrofolate is a substrate of MTHFD2LCompoundRhea RHEA:22892Inferred only
(6R)-5,10-methylene-5,6,7,8-tetrahydrofolate is a substrate of MTHFD2LCompoundRhea RHEA:22812Inferred only
(6R)-5,10-methylenetetrahydrofolic acid is a substrate of MTHFD2LCompoundHuman-GEM MAR04440Inferred only
10-formyltetrahydrofolic acid is a substrate of MTHFD2LCompoundHuman-GEM MAR04505Inferred only
5,6,7,8-tetrahydrofolic acid is a substrate of MTHFD2LCompoundHuman-GEM MAR04340Inferred only
formic acid is a substrate of MTHFD2LCompoundHuman-GEM MAR04340Inferred only

Reactions

6 reactions

ATP+formic acid+5,6,7,8-tetrahydrofolic acid10-formyltetrahydrofolic acid+ADP+phosphate(3-)
Catalysed by MTHFD2 or MTHFD1L or MTHFD2L
Reaction pageLeft to rightBalancedEC 3.5.4.9, 6.3.4.3mitochondrial matrix
Human-GEM MAR04340CC BY 4.0Reference data
(6R)-5,10-methenyltetrahydrofolate+NADH(2-)(6R)-5,10-methylenetetrahydrofolic acid

Other names

3 names

Resolves to: MTHFD2L

Also called: ; Methenyltetrahydrofolate cyclohydrolaseMTHFD2-likeNADP-dependent methylenetetrahydrofolate dehydrogenase 2-like protein

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Catalysed by MTHFD2 or MTHFD2L

Reaction pageReversibleBalancedEC 1.5.1.15mitochondrial matrix

Human-GEM MAR04440CC BY 4.0Reference data

10-formyltetrahydrofolic acid+proton(6R)-5,10-methenyltetrahydrofolate+water

Catalysed by MTHFD2 or MTHFD1L or MTHFD2L

Reaction pageReversibleBalancedEC 3.5.4.9mitochondrial matrix

Human-GEM MAR04505CC BY 4.0Reference data

(6R)-5,10-methylene-5,6,7,8-tetrahydrofolate+NADP(3-)(6R)-5,10-methenyltetrahydrofolate+NADPH(4-)

Catalysed by MTHFD1 or MTHFD2L

Reaction pageLeft to rightBalanced

Rhea RHEA:22812CC BY 4.0Reference data

(6R)-5,10-methylene-5,6,7,8-tetrahydrofolate+NAD(1-)(6R)-5,10-methenyltetrahydrofolate+NADH(2-)

Catalysed by MTHFD2 or MTHFD2L

Reaction pageDirection not assertedBalanced

Rhea RHEA:22892CC BY 4.0Reference data

(6R)-5,10-methenyltetrahydrofolate+water(6R)-10-formyltetrahydrofolate+hydron

Catalysed by MTHFD1 or MTHFD2 or MTHFD2L

Reaction pageDirection not assertedBalanced

Rhea RHEA:23700CC BY 4.0Reference data