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

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

authored	[InstanceEdit:1497960] Jassal, B, 2011-08-17
created	[InstanceEdit:1474141] Jassal, B, 2011-08-04
dbId	1474151
displayName	Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation
edited	[InstanceEdit:1497960] Jassal, B, 2011-08-17
eventOf	[Pathway:R-HSA-8978934] Metabolism of cofactors - Homo sapiens
goBiologicalProcess	[GO_BiologicalProcess:21492] tetrahydrobiopterin metabolic process
hasDiagram	false
hasEHLD	false
hasEvent	[Reaction:R-HSA-1474146] GCH1 reduces GTP to dihydroneopterin triphosphate - Homo sapiens [Reaction:R-HSA-1475422] PTPS is phosphorylated by cGMP-dependant protein kinase II - Homo sapiens [Reaction:R-HSA-1474184] DHNTP is dephosphorylated by PTPS to PTHP - Homo sapiens [Reaction:R-HSA-1497853] Sepiapterin reductase (SPR) is phosphorylated by Ca2+/calmodulin-dependent protein kinase II - Homo sapiens [Reaction:R-HSA-1475414] PTHP is reduced to BH4 by sepiapterin reductase (SPR) - Homo sapiens [BlackBoxEvent:R-HSA-9693722] Unknown sepiapterin synthase transforms PTHP to sepiapterin - Homo sapiens [Reaction:R-HSA-1497824] BH4 is oxidised to the BH3 radical during the eNOS catalytic cycle - Homo sapiens [Reaction:R-HSA-1497866] Peroxynitrite can oxidise BH4 to the BH3 radical - Homo sapiens [Reaction:R-HSA-1497883] Ferrous iron reduces the BH3 radical back to BH4 - Homo sapiens [Reaction:R-HSA-1497855] Ascorbate can reduce the BH3 radical back to BH4 - Homo sapiens [BlackBoxEvent:R-HSA-1497863] The BH3 radical can decay to dihydrobiopterin (BH2) - Homo sapiens [Reaction:R-HSA-1497784] The cofactor BH4 is required for electron transfer in the eNOS catalytic cycle - Homo sapiens [Reaction:R-HSA-1497796] BH2 binding can lead to eNOS uncoupling - Homo sapiens [Reaction:R-HSA-1474158] GCHFR binds to GCH1 and negatively regulates its activity - Homo sapiens [Reaction:R-HSA-1497869] Salvage - Sepiapterin is reduced to q-BH2 - Homo sapiens [Reaction:R-HSA-1497794] Salvage - BH2 is reduced to BH4 by DHFR - Homo sapiens
isInDisease	false
isInferred	false
lastUpdatedDate	2020-09-24
literatureReference	[LiteratureReference:1474130] Tetrahydrobiopterin biosynthesis, regeneration and functions [LiteratureReference:1497795] Mechanisms for the role of tetrahydrobiopterin in endothelial function and vascular disease [LiteratureReference:1497786] Nitric oxide, tetrahydrobiopterin, oxidative stress, and endothelial dysfunction in hypertension [LiteratureReference:1497881] Synthesis and recycling of tetrahydrobiopterin in endothelial function and vascular disease
maxDepth	2
modified	[InstanceEdit:11108703] Weiser, Joel, 2026-03-16
name	Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation
orthologousEvent	[Pathway:R-PFA-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Plasmodium falciparum [Pathway:R-SCE-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Saccharomyces cerevisiae [Pathway:R-SPO-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Schizosaccharomyces pombe [Pathway:R-DDI-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Dictyostelium discoideum [Pathway:R-CEL-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Caenorhabditis elegans [Pathway:R-DME-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Drosophila melanogaster [Pathway:R-GGA-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Gallus gallus [Pathway:R-XTR-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Xenopus tropicalis [Pathway:R-DRE-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Danio rerio [Pathway:R-SSC-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Sus scrofa [Pathway:R-BTA-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Bos taurus [Pathway:R-CFA-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Canis familiaris [Pathway:R-RNO-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Rattus norvegicus [Pathway:R-MMU-1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - Mus musculus
releaseDate	2011-09-20
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:1502545] D'Eustachio, P, 2011-08-23
schemaClass	Pathway
species	[Species:48887] Homo sapiens
stId	R-HSA-1474151
summation	[Summation:1474122] Tetrahydrobiopterin (BH4) is an essential co-factor for the ...
updateTrackers	[UpdateTracker:9776828] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v74:[addHasEvent] [UpdateTracker:9786482] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v49:[modifyText] [UpdateTracker:9780270] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v63:[addHasEvent, updateContainedRLE] [UpdateTracker:9777676] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v69:[updateContainedRLE]

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(updatedInstance)

[UpdateTracker:9776828] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v74:[addHasEvent]
[UpdateTracker:9786482] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v49:[modifyText]
[UpdateTracker:9780270] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v63:[addHasEvent, updateContainedRLE]
[UpdateTracker:9777676] Update Tracker - [Pathway:1474151] Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation - v69:[updateContainedRLE]

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[Pathway:R-HSA-8978934] Metabolism of cofactors

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