Purine metabolism (Homo sapiens)

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128 steps involvedIMPDH1RRUrateXMPGuanineGDPAICA-ribosidedGDPAPRTRibose-5-PInosineITPAdGMPADSLSAICARPGMPPRPS1S-AMP2,8-DihydroxyadeninedATPXanthosinedAMPADSSATICAdeninePRPPHPRT12-Deoxyguanosine2'-DeoxyadenosineSAICA-ribosideADSLGTPAICARPADPFAICARPdADPAMPPRPPsHypoxanthineDGUOKdGTPGuanosinePNPSuccinyladenosineAMPD1ITPXanthineATPADAAdenosine2'-deoxyinosineIMPPNPPNPXORRATICADAPNPHPRT1XOPNPXOPNP


Description

Overview of purine metabolism and related diseases. Disorders resulting from an enzyme defect are highlighted in pink, metabolic markers are highlighted in red. Arrows indicate the directionality of chemical conversions.

On the right, the biosynthesis of IMP is depicted in more detail (adapted from: https://en.wikipedia.org/wiki/Purine_metabolism). The color scheme for this part of the pathway is as follows: enzymes(black), coenzymes(light orange), regular substrates/metabolites(blue), additional substrates(dark green), metal ions(turquoise), inorganic molecules(light purple).

This pathway was inspired by Chapter 41 of the book of Blau (ISBN 3642403360 (978-3642403361)).

Proteins on this pathway have targeted assays available via the CPTAC Assay Portal

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Ontology Terms

 

Bibliography

  1. Su, H, Lenardo, M.; ''Chapter 5 Combined Immune Deficiencies''; https://doi.org/10.1016/B978-0-12-405546-9.00005-4, 2014 DOI Scholia
  2. Blau, N., Duran, M., Gibson, K.M., Dionisi-Vici, C.; ''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases, Chapter 11 and 41''; ISBN 978-3-642-40337-8, 2014

History

View all...
CompareRevisionActionTimeUserComment
134645view12:25, 23 July 2024EweitzNote full pathway with related disorders
134644view12:20, 23 July 2024EweitzModified description
124888view13:57, 29 December 2022EgonwManually added the DOI identifier as identifier too
119117view16:30, 17 June 2021FinterlyAdded ISBN for book citation
109348view12:57, 16 March 2020L DupuisConnected one unconnected line
109308view08:42, 10 March 2020DeSlOntology Term : 'DOID:5810' removed !
109256view15:18, 2 March 2020Mkutmonfixed unconnected lines
109224view09:43, 28 February 2020Mkutmonlayout adaption
108876view08:44, 4 February 2020DeSlModified description
108875view08:43, 4 February 2020DeSlModified description
108874view08:42, 4 February 2020DeSlOntology Term : 'DOID:0080121' removed !
108873view08:41, 4 February 2020DeSlOntology Term : 'PW:0001779' removed !
108870view15:12, 3 February 2020DeSlOntology Term : 'DOID:1919' removed !
108869view15:12, 3 February 2020DeSlOntology Term : 'PW:0001592' removed !
108868view15:12, 3 February 2020DeSlOntology Term : 'DOID:0060350' removed !
108867view15:10, 3 February 2020DeSlOntology Term : 'PW:0001777' removed !
108866view15:09, 3 February 2020DeSlOntology Term : 'DOID:0050762' removed !
108865view15:09, 3 February 2020DeSlOntology Term : 'PW:0001879' removed !
108864view15:08, 3 February 2020DeSlOntology Term : 'PW:0001776' removed !
108863view15:08, 3 February 2020DeSlModified description
108862view15:07, 3 February 2020DeSlModified description
108861view15:05, 3 February 2020DeSlNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
2'-DeoxyadenosineMetaboliteCHEBI:17256 (ChEBI)
2'-deoxyinosineMetaboliteCHEBI:28997 (ChEBI)
2,8-DihydroxyadenineMetaboliteQ4596812 (Wikidata)
2-DeoxyguanosineMetaboliteCHEBI:17172 (ChEBI)
ADAProteinP00813 (Uniprot-TrEMBL)
ADPMetaboliteCHEBI:456216 (ChEBI)
ADSLProteinP30566 (Uniprot-TrEMBL)
ADSSProteinP30520 (Uniprot-TrEMBL) Adenylosuccinate synthetase isozyme 2
AICA-ribosideMetaboliteCHEBI:28498 (ChEBI)
AICARPMetaboliteCHEBI:58475 (ChEBI)
AMPMetaboliteCHEBI:456215 (ChEBI)
AMPD1ProteinP23109 (Uniprot-TrEMBL)
APRTProteinP07741 (Uniprot-TrEMBL)
ATICProteinP31939 (Uniprot-TrEMBL)
ATPMetaboliteCHEBI:30616 (ChEBI)
AdenineMetaboliteCHEBI:16708 (ChEBI)
AdenosineMetaboliteCHEBI:16335 (ChEBI)
DGUOKProteinQ16854 (Uniprot-TrEMBL)
FAICARPMetaboliteCHEBI:58467 (ChEBI)
GDPMetaboliteCHEBI:58189 (ChEBI)
GMPMetaboliteCHEBI:58115 (ChEBI)
GTPMetaboliteCHEBI:37565 (ChEBI)
GuanineMetaboliteCHEBI:16235 (ChEBI)
GuanosineMetaboliteCHEBI:16750 (ChEBI)
HPRT1ProteinP00492 (Uniprot-TrEMBL)
HypoxanthineMetaboliteCHEBI:17368 (ChEBI)
IMPMetaboliteCHEBI:58053 (ChEBI)
IMPDH1ProteinP20839 (Uniprot-TrEMBL)
ITPAProteinQ9BY32 (Uniprot-TrEMBL)
ITPMetaboliteCHEBI:61402 (ChEBI) is a nucleoside triphosphate(4−)
InosineMetaboliteCHEBI:17596 (ChEBI)
PNPProteinP00491 (Uniprot-TrEMBL)
PRPPMetaboliteCHEBI:17111 (ChEBI)
PRPPsProtein2.7.6.1 (Enzyme Nomenclature)
PRPS1ProteinP60891 (Uniprot-TrEMBL)
RRProtein1.17.4.1 (Enzyme Nomenclature)
Ribose-5-PMetaboliteCHEBI:52742 (ChEBI)
S-AMPMetaboliteCHEBI:57567 (ChEBI) succinyladenosine monophosphate
SAICA-ribosideMetaboliteCHEBI:18319 (ChEBI)
SAICARPMetaboliteCHEBI:58443 (ChEBI)
SuccinyladenosineMetaboliteCHEBI:71169 (ChEBI)
UrateMetaboliteCHEBI:17775 (ChEBI)
XMPMetaboliteCHEBI:57464 (ChEBI)
XOProteinP47989 (Uniprot-TrEMBL)
XanthineMetaboliteCHEBI:17712 (ChEBI)
XanthosineMetaboliteCHEBI:18107 (ChEBI)
dADPMetaboliteCHEBI:57667 (ChEBI)
dAMPMetaboliteCHEBI:58245 (ChEBI)
dATPMetaboliteCHEBI:61404 (ChEBI)
dGDPMetaboliteCHEBI:58595 (ChEBI)
dGMPMetaboliteCHEBI:57673 (ChEBI)
dGTPMetaboliteCHEBI:16497 (ChEBI)

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
2'-Deoxyadenosine2'-deoxyinosinemim-conversion28191 (Rhea)
2'-DeoxyadenosineAdeninemim-conversion27743 (Rhea)
2'-deoxyinosineHypoxanthinemim-conversion27751 (Rhea)
2-DeoxyguanosineGuaninemim-conversion27739 (Rhea)
2-DeoxyguanosinedGMPmim-conversion19202 (Rhea)
2-Deoxyguanosinemim-conversion19203 (Rhea)
ADPATPmim-conversion30657 (Rhea)
ADPdADPmim-conversion28036 (Rhea)
AICARPFAICARPmim-conversion22193 (Rhea)
AMPADPmim-conversion11462 (Rhea)
AMPAdenosinemim-conversion29376 (Rhea)
AMPIMPmim-conversion14778 (Rhea)
AdenineAMPmim-conversion16611 (Rhea)
AdenosineAdeninemim-conversion18670 (Rhea)
AdenosineInosinemim-conversion24409 (Rhea)
FAICARPIMPmim-conversion18447 (Rhea)
GDPGTPmim-conversion29865 (Rhea)
GDPdGDPmim-conversion28032 (Rhea)
GMPGDPmim-conversion20781 (Rhea)
GMPGuanosinemim-conversion27715 (Rhea)
Guaninemim-conversion14666 (Rhea)
Guaninemim-conversion25426 (Rhea)
GuanosineGuaninemim-conversion13234 (Rhea)
HypoxanthineXanthinemim-conversion24671 (Rhea)
Hypoxanthinemim-conversion17975 (Rhea)
IMPInosinemim-conversion27719 (Rhea)
IMPS-AMPmim-conversion15754 (Rhea)
IMPXMPmim-conversion11709 (Rhea)
ITPIMPmim-conversion29400 (Rhea)
InosineHypoxanthinemim-conversion16658 (Rhea)
S-AMPAMPmim-conversion16854 (Rhea)
SAICARPAICARPmim-conversion23921 (Rhea)
XMPGMPmim-conversion18302 (Rhea)
XMPXanthosinemim-conversion28531 (Rhea)
XanthineUratemim-conversion16670 (Rhea)
XanthosineXanthinemim-conversion27639 (Rhea)
dADPdAMPmim-conversion23102 (Rhea)
dADPdATPmim-conversion27675 (Rhea)
dAMP2'-Deoxyadenosinemim-conversion29372 (Rhea)
dGDPdGMPmim-conversion12699 (Rhea)
dGDPdGTPmim-conversion27691 (Rhea)
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