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: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 MoleculeUreaend product of the Urea cycle pathway

Pathway Gene Annotations

Disease Annotations Associated with Genes in the urea cycle pathway
Disease TermsGene Symbols
Drug ToxicityAss1
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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