UREA CYCLE PATHWAY (PW:0000076)

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Description

The urea cycle, the first known metabolic cycle, was elucidated by Krebs and Henseleit in 1932. It is composed of seven gene products, and operates predominantly in mammals to eliminate excess nitrogen arising from the breakdown of amino acids through the synthesis in the liver of urea, which is then excreted, while the amino acid carbon skeletons are converted to glucose or glycogen. Mitochondrial carbamoyl-phosphate synthetase (Cps1) condenses ammonia from amino acid catabolism, the first of u

Pathway Diagram:

Elsevier Inc. Ass1 Arg1 Otc Nos NO ornithine fumarate arginine argininosuccinate aspartate NH4+ citrulline carbamoyl phosphate N-acetylglutamate urea Nags acetyl-CoA glutamate NADPH O2 H+ H2O NADP+ amino acid metabolic pathway N-acetylglutamate ---> Cps1 amino acid metabolic pathway ---> NH4+ AMP ATP PPi H2O 2 ATP 2 ADP Pi HCO3- Slc25a15 Pi ornithine ---> citrulline ATP ---> AMP aspartate ---> argininosuccinate citrulline ---> argininosuccinate H2O ---> urea Cps1 Asl NADPH ---> NO arginine ---> citrulline argininosuccinate ---> arginine argininosuccinate ---> fumarate HCO3- ---> carbamoyl phosphate glutamate ---> N-acetylglutamate carbamoyl phosphate ---> Pi arginine ---> ornithine
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Genes in Pathway:

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urea cycle pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Arg1 arginase 1 JBrowse link 1 21,525,421 21,537,872 RGD:2300098
RGD:10402751
G Arg2 arginase 2 JBrowse link 6 102,311,097 102,338,406 RGD:4144088
G Asl argininosuccinate lyase JBrowse link 12 30,160,922 30,178,348 RGD:2300098
RGD:10402751
G Ass1 argininosuccinate synthase 1 JBrowse link 3 10,327,411 10,375,847 RGD:2300098
RGD:10402751
G Cps1 carbamoyl-phosphate synthase 1 JBrowse link 9 74,113,437 74,236,274 RGD:1582379
RGD:10402751
RGD:2300098
G Gls2 glutaminase 2 JBrowse link 7 2,605,679 2,621,853 RGD:10402751
G Glud1 glutamate dehydrogenase 1 JBrowse link 16 10,661,486 10,695,557 RGD:10402751
G Got2 glutamic-oxaloacetic transaminase 2 JBrowse link 19 9,587,637 9,613,323 RGD:10402751
G Gpt glutamic--pyruvic transaminase JBrowse link 7 117,759,083 117,761,932 RGD:10402751
G Otc ornithine carbamoyltransferase JBrowse link X 13,524,804 13,601,074 RGD:1582379
RGD:10402751
RGD:2300098
G Slc1a4 solute carrier family 1 member 4 JBrowse link 14 104,582,884 104,612,417 RGD:10402751
G Slc1a5 solute carrier family 1 member 5 JBrowse link 1 78,710,686 78,724,789 RGD:10402751
G Slc25a12 solute carrier family 25 member 12 JBrowse link 3 57,881,951 57,998,214 RGD:10402751
G Slc25a15 solute carrier family 25 member 15 JBrowse link 16 74,505,318 74,554,523 RGD:10402751

Additional Elements in Pathway:

(includes Gene Groups, Small Molecules, Other Pathways..etc.)
Object TypePathway ObjectPathway Object Description
Small MoleculeNitric oxideend product of the arginine-citrulline cycle
Small MoleculeFumarateend product of the urea cycle pathway
Small MoleculeUreaend product of the urea cycle pathway
Gene GroupNitric oxide synthasesenzyme of the arginine-citrulline cycle
Small MoleculeAmmoniuminput molecule of the urea cycle pathway
Small MoleculeAspartateinput molecule of the urea cycle pathway
Small MoleculeCitrullineintermediate of the urea cycle pathway
Small MoleculeOrnithineintermediate of the urea cycle pathway
Small MoleculeArginineintermediate of the urea cycle pathway
Small MoleculeArginosuccinateintermediate of the urea cycle pathway
Small MoleculeN-acetylglutamateregulatory molecule of the urea cycle pathway

Pathway Gene Annotations

Disease Annotations Associated with Genes in the urea cycle pathway
Disease TermsGene Symbols
AcidosesCps1 , Otc
acute kidney failureArg1 , Arg2 , Got2
Acute Necrotizing PancreatitisArg1
Acute-Phase ReactionAss1
Alcoholic Liver DiseasesOtc
Alzheimer's diseaseAss1 , Glud1
amino acid metabolic disorderArg1 , Asl , Slc25a15
Animal Disease ModelsArg1
Animal HepatitisCps1 , Otc
argininosuccinic aciduriaAsl
asbestosisArg1
Asperger syndromeSlc25a12
asthmaArg1 , Arg2
autism spectrum disorderSlc25a12
autistic disorderOtc , Slc25a12
brain diseaseOtc
brain ischemiaGlud1
Canavan diseaseGlud1
carbamoyl phosphate synthetase I deficiency diseaseCps1
CARBONIC ANHYDRASE VA DEFICIENCY, HYPERAMMONEMIA DUE TOCps1
Chemical and Drug Induced Liver InjuryArg1 , Cps1 , Gpt
childhood absence epilepsyGlud1
cholestasisArg1 , Otc
citrullinemiaAss1 , Slc25a15
CITRULLINEMIA TYPE 2Arg1
cognitive disorderOtc
ComaCps1
Congenital HyperinsulinismGlud1
Developmental DisabilitiesOtc
diabetes mellitusGlud1
Diabetes Mellitus, Experimental Arg1 , Arg2 , Ass1 , Cps1 , Otc
diabetic angiopathyAss1
Diabetic NephropathiesArg2
Disease ProgressionArg2
early infantile epileptic encephalopathy 39Slc25a12
end stage renal failureArg2
EndotoxemiaAsl , Ass1 , Cps1 , Otc
Experimental Autoimmune EncephalomyelitisGlud1
Experimental Liver CirrhosisArg1 , Arg2 , Ass1 , Cps1 , Gls2 , Gpt , Otc
Experimental Liver NeoplasmsCps1
familial hyperinsulinemic hypoglycemia 6Glud1
fascioliasisGlud1
fatty liver diseaseCps1 , Gpt , Otc
Febrile SeizuresGot2
Fetal Growth RetardationArg1 , Cps1 , Glud1 , Otc
genetic diseaseOtc , Slc1a4
hair diseaseArg1
hepatic encephalopathyGlud1
hepatocellular carcinomaArg1 , Ass1
HyperammonemiaAss1 , Cps1 , Glud1 , Otc , Slc25a15
hyperargininemiaArg1
HypercapniaArg1
hyperinsulinismGlud1
HyperoxiaArg1 , Arg2 , Asl , Ass1
hypertensionArg1 , Arg2 , Cps1
hypoglycemiaGlud1
HypoxiaAsl , Ass1
Immediate HypersensitivityArg1 , Arg2
immune system diseaseArg1
Insulin ResistanceArg1
intellectual disabilitySlc25a15
Intestinal FistulaArg1
juvenile polyposis syndromeGlud1
leishmaniasisArg1
liver diseaseArg1 , Gpt
Liver FailureArg1 , Asl , Ass1 , Cps1 , Otc
lung cancerCps1
melanomaAss1
Mental Retardation, Autosomal Recessive 18Arg1
muscular diseaseArg1
NecrosisArg2
Neonatal Pulmonary HypertensionCps1
Nerve DegenerationOtc
neurodegenerative diseaseGot2
obesityCps1 , Got2 , Otc
ornithine carbamoyltransferase deficiencyOtc
ornithine translocase deficiencySlc25a15
pancreatic ductal carcinomaArg2
persistent fetal circulation syndromeCps1
pleomorphic xanthoastrocytomaAsl
pre-malignant neoplasmArg1 , Cps1
Primary Hyperoxaluria Type 1Gpt
Prostatic NeoplasmsArg2
pulmonary hypertensionArg2 , Cps1
Pulmonary Hypertension, Hypoxia-Induced Got2
Reperfusion InjuryArg1 , Asl , Ass1 , Glud1 , Gpt , Otc
rheumatoid arthritisSlc25a12
schizophreniaGot2
SepsisArg2 , Otc
Shock, HemorrhagicArg2 , Otc
silicosisArg1
SPASTIC TETRAPLEGIA, THIN CORPUS CALLOSUM, AND PROGRESSIVE MICROCEPHALYSlc1a4
Spinal Cord InjuriesGot2
Sudden DeathOtc
temporal lobe epilepsyGlud1
Transplant RejectionGot2
trichorhinophalangeal syndrome type ICps1
type 2 diabetes mellitusAsl
ureteral obstructionArg1 , Arg2
uveitisArg1 , Ass1
vascular diseaseCps1
Veno-Occlusive DiseaseCps1
visual epilepsyGlud1
Wounds and InjuriesArg1
Pathway Annotations Associated with Genes in the urea cycle pathway
Pathway TermsGene Symbols
2-hydroxyglutaric aciduria pathwayCps1 , Gls2 , Glud1 , Got2 , Gpt
AGAT deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
alanine metabolic pathwayGpt
alanine, aspartate and glutamate metabolic pathwayAsl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt
arginine and proline metabolic pathwayArg1 , Arg2 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Otc , Slc25a15
argininosuccinic aciduria pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
Canavan disease pathwayAsl , Ass1
carbamoyl phosphate synthetase I deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
citrullinemia pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
cysteine and methionine metabolic pathwayGot2
D-glutamine and D-glutamate metabolic pathwayGls2 , Glud1
Entamoebiasis pathwayArg1 , Arg2
gluconeogenesis pathwayGot2
glutamate signaling pathwayGls2
glutamic acid/glutamate metabolic pathwayCps1 , Gls2 , Glud1 , Got2 , Gpt
guanidinoacetate methyltransferase deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
gyrate atrophy pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
homocarnosinosis pathwayCps1 , Gls2 , Glud1 , Got2 , Gpt
hyperargininemia pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
hyperprolinemia type I pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
hyperprolinemia type II pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
interleukin-4 signaling pathwayArg1
kynurenine metabolic pathwayGot2
lactic acidosis pathwayGpt
nicotinamide adenine dinucleotide metabolic pathwayGot2 , Slc25a12
ornithine carbamoyltransferase deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
ornithine translocase deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
phenylalanine metabolic pathwayGot2
phenylalanine, tyrosine and tryptophan biosynthetic pathwayGot2
primary hyperoxaluria type 1 pathwayGpt
prolidase deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
pyruvate carboxylase deficiency pathwayGpt
succinic semialdehyde dehydrogenase deficiency pathwayCps1 , Gls2 , Glud1 , Got2 , Gpt
tyrosine metabolic pathwayGot2
urea cycle pathwayArg1 , Arg2 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15

References Associated with the urea cycle pathway:

Ontology Path Diagram:

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