Urea cycle and metabolism of amino groups (Homo sapiens)

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N-MethylhydantoinSMS2.7.3.1CPS13.5.2.14(S)-1-Pyrroline-5-carboxylateGlutamate metabolism3.5.2.10N-Acetyl-glutamateAmino acid metabolismL-GlutamateArginine3.5.3.3Proline metabolismCKMcreatine-PL-GlutamylNAGSPurine metabolismACY1ASSNH33.5.1.5Urea2.6.1.11ALDH18A1L-Glutamate 5-semialdehydeN-Carbamoylsarcosine2.3.1.35OATUrea-1-carboxylateN2-Acetyl-L-ornithineGAMTNitrogen metabolismASL3.5.1.16Pyrimidine metabolismN-Acetyl-L-glutamateODC1D-Arginine and D-Ornithine metabolism6.3.4.6Alanine and aspartate metabolismCKBFumarate2.7.2.8L-AspartateGuanidinoacetate1. cycle (TCA cycle)SpermidineCarbamoylCreatinineL-ProlineCO21.2.1.46PhosphoguanidinoacetateFormateSpermineSMSN-Acetyl-L-glutamateALDH18A1Arginine metabolismPutrescinePYCRL


No description


GenMAPP notes 
Converted from rat to human using ortholog information. Link to original KEGG map: http://www.genome.ad.jp/dbget-bin/show pathway?rno00220
GenMAPP remarks 
Edited by Sebastien Burel

Quality Tags

Ontology Terms



  1. Dahlman, Douglas L., Milan A. Berge.; ''Possible mechanisms for adverse effects of L-canavanine on insects.''; ACS Publications, 1986
  2. Seiler N; ''Ornithine aminotransferase, a potential target for the treatment of hyperammonemias.''; Curr Drug Targets, 2000 PubMed Europe PMC Scholia
  3. Deuschle K, Funck D, Hellmann H, Däschner K, Binder S, Frommer WB; ''A nuclear gene encoding mitochondrial Delta-pyrroline-5-carboxylate dehydrogenase and its potential role in protection from proline toxicity.''; Plant J, 2001 PubMed Europe PMC Scholia
  4. Dahlman, Douglas L., Milan A. Berge.; ''Possible mechanisms for adverse effects of L-canavanine on insects.''; , 1986


View all...
105850view23:15, 15 August 2019KhanspersModified description
103837view12:35, 10 April 2019IreneHemelAdded Uniprot IDs
103836view11:00, 10 April 2019IreneHemelChanged datanodes for enzymes from geneproduct to protein
96019view15:13, 15 February 2018Mkutmonremoved old pathways attributes
96018view15:13, 15 February 2018MkutmonModified description
95281view18:12, 29 November 2017DeSlChanged several graphical lines to directed interactions + added litertaure references for these.
94180view13:40, 25 August 2017DeSlAdded ID for L-Glutamyl
92933view09:00, 20 July 2017EgonwFixed to the correct CAS number: it had the number of creatine.
72142view10:02, 25 October 2013Mkutmonadded datasource to EC numbers
71261view21:34, 16 October 2013MaintBotAutomated update of data sources
71169view11:01, 11 October 2013Mkutmonfixed unconnected lines
68976view17:41, 8 July 2013MaintBotUpdated to 2013 gpml schema
45014view18:23, 6 October 2011KhanspersOntology Term : 'urea cycle pathway' added !
43586view14:08, 5 July 2011AdrienDefayadd database name for TCA
41149view23:40, 1 March 2011MaintBotRemoved redundant pathway information and comments
38842view17:40, 24 September 2010Khanspers
38688view18:15, 23 September 2010KhanspersMore pathway links
38687view18:08, 23 September 2010KhanspersAdded pathway links and changed arrows and layout
38685view18:04, 23 September 2010KhanspersPeriodical save, work in progress
34435view18:15, 10 December 2009MaintBotAutomatic update of empty xrefs
32123view13:53, 14 August 2009MaintBotFixed group labels
21724view11:32, 14 November 2008MaintBot[[Pathway:Homo sapiens:Urea cycle and metabolism of amino groups KEGG]] moved to [[Pathway:WP497]]: Moved to stable identifier
13997view16:08, 20 May 2008Thomasconnected lines
13129view11:18, 17 May 2008MaintBotautomated metabolite conversion
8476view13:46, 7 January 2008MaintBot[[Pathway:Human:Urea cycle and metabolism of amino groups KEGG]] moved to [[Pathway:Homo sapiens:Urea cycle and metabolism of amino groups KEGG]]: Renaming species
7854view16:23, 18 December 2007MaintBotfixed category names
7413view12:42, 4 November 2007MaintBotAdded categories to GPML
6404view22:18, 22 May 2007L.M.Ferrantegpml file for [[Human:Urea_cycle_and_metabolism_of_amino_groups_KEGG]]

External references


View all...
NameTypeDatabase referenceComment
(S)-1-Pyrroline-5-carboxylateMetaboliteC03912 (Kegg Compound)
ACY1GeneProduct95 (Entrez Gene)
ALDH18A1GeneProduct5832 (Entrez Gene)
ARG1GeneProduct383 (Entrez Gene)
ARG2GeneProduct384 (Entrez Gene)
ASLGeneProduct435 (Entrez Gene)
ASSGeneProduct445 (Entrez Gene)
Alanine and aspartate metabolismPathwayWP106
Amino acid metabolismPathway
Arginine metabolismPathway
ArginineMetabolite74-79-3 (CAS) L-Arginine
CKBGeneProduct1152 (Entrez Gene)
CKMGeneProduct1158 (Entrez Gene)
CO2MetaboliteC00011 (Kegg Compound)
CPS1GeneProduct1373 (Entrez Gene)
CarbamoylMetaboliteC00169 (Kegg Compound)
Citrate cycle (TCA cycle)PathwayWP78 (WikiPathways)
CreatineMetabolite57-00-1 (CAS) Creatine
CreatinineMetabolite60-27-5 (CAS) Creatinine
D-Arginine and D-Ornithine metabolismPathway
FormaldehydeMetabolite50-00-0 (CAS) Formaldehyde
FormateMetaboliteC00058 (Kegg Compound)
FumarateMetaboliteC00122 (Kegg Compound)
GAMTGeneProduct2593 (Entrez Gene)
GATMGeneProduct2628 (Entrez Gene)
GLUD1GeneProduct2746 (Entrez Gene)
Glutamate metabolismPathway
GuanidinoacetateMetaboliteC00581 (Kegg Compound)
L-AspartateMetaboliteC00049 (Kegg Compound)
L-CitrullineMetabolite372-75-8 (CAS) L-Citrulline
L-Glutamate 5-semialdehydeMetaboliteC01165 (Kegg Compound)
L-GlutamateMetabolite56-86-0 (CAS) L-Glutamate
L-OrnithineMetabolite70-26-8 (CAS) L-Ornithine
L-ProlineMetabolite147-85-3 (CAS) L-Proline
N-(L-Arginino)succinateMetaboliteC03406 (Kegg Compound)
N-Acetyl-L-glutamateMetaboliteC00624 (Kegg Compound)
N-Acetyl-glutamateMetaboliteC00624 (Kegg Compound)
N-CarbamoylsarcosineMetaboliteC01043 (Kegg Compound)
N-MethylhydantoinMetaboliteC02565 (Kegg Compound)
N2-Acetyl-L-ornithineMetaboliteC00437 (Kegg Compound)
NAGSGeneProduct162417 (Entrez Gene)
NH3MetaboliteC00014 (Kegg Compound)
Nitrogen metabolism Pathway
OATGeneProduct4942 (Entrez Gene)
ODC1GeneProduct4953 (Entrez Gene)
OTCGeneProduct5009 (Entrez Gene)
PYCR1GeneProduct5831 (Entrez Gene)
PYCRLGeneProduct65263 (Entrez Gene)
PhosphoguanidinoacetateMetaboliteC03166 (Kegg Compound)
Proline metabolismPathway
Purine metabolismPathway
PutrescineMetabolite110-60-1 (CAS) Putrescine
Pyrimidine metabolismPathway
SMSGeneProduct6611 (Entrez Gene)
SRMGeneProduct6723 (Entrez Gene)
SarcosineMetabolite107-97-1 (CAS) Sarcosine
SpermidineMetabolite124-20-9 (CAS) Spermidine
SpermineMetabolite71-44-3 (CAS) Spermine
Urea-1-carboxylateMetaboliteC01010 (Kegg Compound)
UreaMetabolite57-13-6 (CAS) Urea
creatine-PMetabolite57-00-1 (CAS) Phosphocreatine

Annotated Interactions

No annotated interactions
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