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 five enzymes, and operates 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. Technically not a member of the urea cycle, mitochondrial carbamoyl-phosphate synthetase (CPS I) condenses ammonia, the first of urea's two nitrogens, and HCO3- to form carbamoyl phospha

Pathway Diagram:

Ariadne Genomics Inc. . Asl Argininosuccinate ---> Arginine Arg1 Cps1 Otc Ass1 Citrulline Pi Carbamoyl-P ---> Pi Carbamoyl-P HCO3- ---> Carbamoyl-P HCO3- NH3 Aspartate Ornithine Arginine Argininosuccinate Fumarate Argininosuccinate ---> Fumarate 2 ADP Ornithine ---> Citrulline ATP ---> AMP ATP PPi AMP Aspartate ---> Argininosuccinate Citrulline ---> Argininosuccinate Arginine ---> Ornithine H20 2 ATP Urea H20 ---> Urea
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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:2300098
RGD:10402751
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:2300098
RGD:10402751
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 MoleculeUreaend product of the Urea cycle pathway

Pathway Gene Annotations

Disease Annotations Associated with Genes in the urea cycle pathway
Disease TermsGene Symbols
AcidosisCps1 , Otc
Acute Kidney InjuryArg1 , Arg2
Acute-Phase ReactionAss1
Alzheimer DiseaseAss1 , Glud1
Amino Acid Metabolism, Inborn ErrorsArg1 , Asl , Cps1 , Slc25a15
AnoxiaAsl , Ass1
Argininosuccinic AciduriaAsl
Arthritis, RheumatoidSlc25a12
AsthmaArg1 , Arg2
Autism Spectrum DisorderSlc25a12
Autistic DisorderSlc25a12
Brain DiseasesOtc
Brain IschemiaGlud1
Canavan DiseaseGlud1
Carbamoyl-Phosphate Synthase I Deficiency DiseaseCps1
Carcinoma, HepatocellularArg1 , Ass1
Carcinoma, Pancreatic DuctalArg2
CholestasisArg1 , Otc
CitrullinemiaAss1 , Slc25a15
Cognition DisordersOtc
ComaCps1
Congenital HyperinsulinismGlud1
Death, SuddenOtc
Diabetes Mellitus, ExperimentalArg1 , Arg2 , Ass1 , Cps1 , Otc
Diabetes Mellitus, Type 2Asl
Diabetic AngiopathiesAss1
Diabetic NephropathiesArg2
Disease ProgressionArg2
Drug ToxicityAss1
Drug-Induced Liver InjuryArg1 , Gpt
Encephalomyelitis, Autoimmune, ExperimentalGlud1
EndotoxemiaAsl , Ass1 , Cps1 , Otc
Epilepsy, AbsenceGlud1
Epilepsy, Temporal LobeGlud1
FascioliasisGlud1
Fatty LiverCps1 , Gpt , Otc
Fetal Growth RetardationCps1 , Glud1 , Otc
Hair DiseasesArg1
Hepatic EncephalopathyGlud1
Hepatitis, AnimalCps1 , Otc
HHH syndromeSlc25a15
HyperammonemiaAss1 , Cps1 , Glud1 , Otc , Slc25a15
HyperargininemiaArg1
HypercapniaArg1
Hyperinsulinemic Hypoglycemia, Familial, 6Glud1
HyperinsulinismGlud1
HyperoxiaArg1 , Arg2 , Asl , Ass1
Hypersensitivity, ImmediateArg1 , Arg2
HypertensionArg1 , Arg2 , Cps1
Hypertension, PulmonaryArg2
HypoglycemiaGlud1
Hypomyelination, Global CerebralSlc25a12
Immune System DiseasesArg1
Insulin ResistanceArg1
Intestinal FistulaArg1
Kidney Failure, ChronicArg2
LeishmaniasisArg1
Liver Cirrhosis, ExperimentalArg1 , Arg2 , Ass1 , Cps1 , Gls2 , Gpt , Otc
Liver DiseasesArg1 , Gpt
Liver Diseases, AlcoholicOtc
Liver FailureArg1 , Asl , Ass1 , Cps1 , Otc
Liver Neoplasms, ExperimentalCps1
MelanomaAss1
Muscular DiseasesArg1
NecrosisArg2
Nerve DegenerationOtc
ObesityCps1 , Otc
Ornithine Carbamoyltransferase Deficiency DiseaseOtc
Pancreatitis, Acute NecrotizingArg1
Persistent Fetal Circulation SyndromeCps1
Precancerous ConditionsArg1 , Cps1
Prostatic NeoplasmsArg2
Pulmonary Hypertension, NeonatalCps1
Reperfusion InjuryArg1 , Asl , Ass1 , Glud1 , Gpt , Otc
SeizuresGlud1
SepsisArg2 , Otc
Shock, HemorrhagicArg2 , Otc
SilicosisArg1
Ureteral ObstructionArg1 , Arg2
UveitisArg1 , Ass1
Vascular DiseasesCps1
Veno-Occlusive DiseaseCps1
Wounds and InjuriesArg1
Pathway Annotations Associated with Genes in the urea cycle pathway
Pathway TermsGene Symbols
2-hydroxyglutaricaciduria disease pathwayCps1 , Gls2 , Glud1 , Got2 , Gpt
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
arginine:glycine amidinotransferase deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
argininosuccinic aciduria disease pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
Canavan disease pathwayAsl , Ass1
carbamoyl phosphate synthase I deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
citrullinemia disease 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
Entamoeba histolyca infection 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 disease pathwayCps1 , Gls2 , Glud1 , Got2 , Gpt
hyperargininemia disease pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
hyperornithinemia-hyperammonemia-homocitrullinemia syndrome pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
hyperprolinemia disease pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
hyperprolinemia type II disease 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 disease pathwayArg1 , Asl , Ass1 , Cps1 , Gls2 , Glud1 , Got2 , Gpt , Otc , Slc1a4 , Slc1a5 , Slc25a12 , Slc25a15
phenylalanine metabolic pathwayGot2
phenylalanine, tyrosine and tryptophan biosynthetic pathwayGot2
primary hyperoxaluria type 1 disease pathwayGpt
prolidase deficiency pathwayArg1 , Asl , Ass1 , Cps1 , Glud1 , Otc , Slc25a15
pyruvate carboxylase deficiency disease 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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