Reactome | Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation

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Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation Show undefined attributes

created	[InstanceEdit:9984900] Weiser, Joel, 2026-03-16
dbId	10100703
displayName	Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation
eventOf	[Pathway:R-MMU-8978934] Metabolism of cofactors - Mus musculus
evidenceType	[EvidenceType:9984902] inferred by electronic annotation
goBiologicalProcess	[GO_BiologicalProcess:21492] tetrahydrobiopterin metabolic process
hasDiagram	false
hasEHLD	false
hasEvent	[Reaction:R-MMU-1474146] GCH1 reduces GTP to dihydroneopterin triphosphate - Mus musculus [Reaction:R-MMU-1475422] PTPS is phosphorylated by cGMP-dependant protein kinase II - Mus musculus [Reaction:R-MMU-1474184] DHNTP is dephosphorylated by PTPS to PTHP - Mus musculus [Reaction:R-MMU-1497853] Sepiapterin reductase (SPR) is phosphorylated by Ca2+/calmodulin-dependent protein kinase II - Mus musculus [Reaction:R-MMU-1475414] PTHP is reduced to BH4 by sepiapterin reductase (SPR) - Mus musculus [Reaction:R-MMU-1497784] The cofactor BH4 is required for electron transfer in the eNOS catalytic cycle - Mus musculus [Reaction:R-MMU-1497796] BH2 binding can lead to eNOS uncoupling - Mus musculus [Reaction:R-MMU-1474158] GCHFR binds to GCH1 and negatively regulates its activity - Mus musculus [Reaction:R-MMU-1497869] Salvage - Sepiapterin is reduced to q-BH2 - Mus musculus
inferredFrom	[Pathway:R-HSA-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Homo sapiens
isInDisease	false
isInferred	true
maxDepth	2
name	Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation
releaseDate	2026-03-25
schemaClass	Pathway
species	[Species:48892] Mus musculus
stId	R-MMU-1474151
summation	[Summation:9984901] This event has been computationally inferred from an event that has been demonstrated in another species. The inference is based on the homology mapping from PANTHER. Briefly, reactions for which all involved PhysicalEntities (in input, output and catalyst) have a mapped orthologue/paralogue (for complexes at least 75% of components must have a mapping) are inferred to the other species. High level events are also inferred for these events to allow for easier navigation. More details and caveats of the event inference in Reactome. For details on PANTHER see also: http://www.pantherdb.org/about.jsp

Referrals

(hasEvent)	[Pathway:R-MMU-8978934] Metabolism of cofactors
(inferredTo)	[Pathway:R-HSA-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation

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