Pathways > Urea cycle and associated pathways
Page last updated: 2024-10-06

Urea cycle and associated pathways

Proteins (24)

ProteinSynonymsTaxonomy
Aspartate aminotransferase, mitochondrialmAspAT; EC 2.6.1.1; EC 2.6.1.7; Fatty acid-binding protein; FABP-1; Glutamate oxaloacetate transaminase 2; Kynurenine aminotransferase 4; Kynurenine aminotransferase IV; Kynurenine--oxoglutarate transaminase 4; Kynurenine--oxoglutarate transaminase IV; PlHomo sapiens (human)
Glutaminase liver isoform, mitochondrialGLS; EC 3.5.1.2; L-glutaminase; L-glutamine amidohydrolaseHomo sapiens (human)
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrialP5C dehydrogenase; EC 1.2.1.88; Aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenaseHomo sapiens (human)
Nitric oxide synthase, inducibleEC 1.14.13.39; Hepatocyte NOS; HEP-NOS; Inducible NO synthase; Inducible NOS; iNOS; NOS type II; Peptidyl-cysteine S-nitrosylase NOS2Homo sapiens (human)
Alanine aminotransferase 1ALT1; EC 2.6.1.2; Glutamate pyruvate transaminase 1; GPT 1; Glutamic--alanine transaminase 1; Glutamic--pyruvic transaminase 1Homo sapiens (human)
Nitric oxide synthase, brainEC 1.14.13.39; Constitutive NOS; NC-NOS; NOS type I; Neuronal NOS; N-NOS; nNOS; Peptidyl-cysteine S-nitrosylase NOS1; bNOSHomo sapiens (human)
Pyrroline-5-carboxylate reductase 1, mitochondrialP5C reductase 1; P5CR 1; EC 1.5.1.2Homo sapiens (human)
Ornithine aminotransferase, mitochondrialEC 2.6.1.13; Ornithine delta-aminotransferase; Ornithine--oxo-acid aminotransferaseHomo sapiens (human)
L-lactate dehydrogenase B chainLDH-B; EC 1.1.1.27; LDH heart subunit; LDH-H; Renal carcinoma antigen NY-REN-46Homo sapiens (human)
Aspartate aminotransferase, cytoplasmiccAspAT; EC 2.6.1.1; EC 2.6.1.3; Cysteine aminotransferase, cytoplasmic; Cysteine transaminase, cytoplasmic; cCAT; Glutamate oxaloacetate transaminase 1; Transaminase AHomo sapiens (human)
Carbamoyl-phosphate synthase [ammonia], mitochondrialEC 6.3.4.16; Carbamoyl-phosphate synthetase I; CPSase IHomo sapiens (human)
Arginase-1EC 3.5.3.1; Liver-type arginase; Type I arginaseHomo sapiens (human)
Nitric oxide synthase, endothelialEC 1.14.13.39; Constitutive NOS; cNOS; EC-NOS; Endothelial NOS; eNOS; NOS type III; NOSIIIHomo sapiens (human)
Malate dehydrogenase, mitochondrialEC 1.1.1.37Homo sapiens (human)
Ornithine transcarbamylase, mitochondrialOTCase; EC 2.1.3.3; Ornithine carbamoyltransferase, mitochondrialHomo sapiens (human)
Malate dehydrogenase, cytoplasmicEC 1.1.1.37; Cytosolic malate dehydrogenase; Diiodophenylpyruvate reductase; 1.1.1.96Homo sapiens (human)
Calcium-binding mitochondrial carrier protein Aralar2Citrin; Mitochondrial aspartate glutamate carrier 2; Solute carrier family 25 member 13Homo sapiens (human)
Argininosuccinate synthaseEC 6.3.4.5; Citrulline--aspartate ligaseHomo sapiens (human)
N-acetylglutamate synthase, mitochondrialEC 2.3.1.1; Amino-acid acetyltransferaseHomo sapiens (human)
Glutamate dehydrogenase 1, mitochondrialGDH 1; EC 1.4.1.3Homo sapiens (human)
Calcium-binding mitochondrial carrier protein Aralar1Mitochondrial aspartate glutamate carrier 1; Solute carrier family 25 member 12Homo sapiens (human)
Argininosuccinate lyaseASAL; EC 4.3.2.1; ArginosuccinaseHomo sapiens (human)
Fumarate hydratase, mitochondrialFumarase; HsFH; EC 4.2.1.2Homo sapiens (human)
Mitochondrial ornithine transporter 1Solute carrier family 25 member 15Homo sapiens (human)

Compounds (20)

CompoundDescription
ureaA compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids.
Nitric OxideA free radical gas produced endogenously by a variety of mammalian cells, synthesized from ARGININE by NITRIC OXIDE SYNTHASE. Nitric oxide is one of the ENDOTHELIUM-DEPENDENT RELAXING FACTORS released by the vascular endothelium and mediates VASODILATION. It also inhibits platelet aggregation, induces disaggregation of aggregated platelets, and inhibits platelet adhesion to the vascular endothelium. Nitric oxide activates cytosolic GUANYLATE CYCLASE and thus elevates intracellular levels of CYCLIC GMP.
ArginineAn essential amino acid that is physiologically active in the L-form.
Ammonium
Aspartate
fumaratesCompounds based on fumaric acid.
uridineA ribonucleoside in which RIBOSE is linked to URACIL.
hydrogen carbonate
Pyruvic AcidAn intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
uracilOne of four nucleotide bases in the nucleic acid RNA.
delta-1-pyrroline-5-carboxylate(L)-isomer is the biologically active form; RN given refers to cpd without isomeric designation; structure
alpha-Ketoglutarate
OxaloacetateAn anionic form of oxaloacetic acid.
Carbamyl PhosphateThe monoanhydride of carbamic acid with PHOSPHORIC ACID. It is an important intermediate metabolite and is synthesized enzymatically by CARBAMYL-PHOSPHATE SYNTHASE (AMMONIA) and CARBAMOYL-PHOSPHATE SYNTHASE (GLUTAMINE-HYDROLYZING).
glutamate
polyglutamineRN given for (D)-isomer
Uridine Monophosphate5'-Uridylic acid. A uracil nucleotide containing one phosphate group esterified to the sugar moiety in the 2', 3' or 5' position.
Acetyl Coenzyme AAcetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
orotidylic acidstructure
orotidineRN given refers to parent cpd; structure
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