Page last updated: 2024-10-06

Urea cycle and metabolism of amino groups

Proteins (21)

ProteinSynonymsTaxonomy
Delta-1-pyrroline-5-carboxylate synthaseP5CS; Aldehyde dehydrogenase family 18 member A1Homo 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)
Creatine kinase M-typeEC 2.7.3.2; Creatine kinase M chain; Creatine phosphokinase M-type; CPK-M; M-CKHomo sapiens (human)
Ornithine decarboxylaseODC; EC 4.1.1.17Homo sapiens (human)
Creatine kinase B-typeEC 2.7.3.2; Brain creatine kinase; B-CK; Creatine kinase B chain; Creatine phosphokinase B-type; CPK-BHomo sapiens (human)
Spermidine synthaseSPDSY; EC 2.5.1.16; Putrescine aminopropyltransferaseHomo sapiens (human)
Carbamoyl-phosphate synthase [ammonia], mitochondrialEC 6.3.4.16; Carbamoyl-phosphate synthetase I; CPSase IHomo sapiens (human)
Guanidinoacetate N-methyltransferaseEC 2.1.1.2Homo sapiens (human)
Arginase-1EC 3.5.3.1; Liver-type arginase; Type I arginaseHomo sapiens (human)
Arginase-2, mitochondrialEC 3.5.3.1; Arginase II; Kidney-type arginase; Non-hepatic arginase; Type II arginaseHomo sapiens (human)
Ornithine transcarbamylase, mitochondrialOTCase; EC 2.1.3.3; Ornithine carbamoyltransferase, mitochondrialHomo sapiens (human)
Spermine synthaseSPMSY; EC 2.5.1.22; Spermidine aminopropyltransferaseHomo sapiens (human)
Argininosuccinate synthaseEC 6.3.4.5; Citrulline--aspartate ligaseHomo sapiens (human)
Pyrroline-5-carboxylate reductase 3P5C reductase 3; P5CR 3; EC 1.5.1.2; Pyrroline-5-carboxylate reductase-like proteinHomo sapiens (human)
Sarcosine dehydrogenase, mitochondrialSarDH; EC 1.5.8.3; BPR-2Homo sapiens (human)
Aminoacylase-1ACY-1; EC 3.5.1.14; N-acyl-L-amino-acid amidohydrolaseHomo 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)
Argininosuccinate lyaseASAL; EC 4.3.2.1; ArginosuccinaseHomo sapiens (human)
Glycine amidinotransferase, mitochondrialEC 2.1.4.1; L-arginine:glycine amidinotransferase; TransamidinaseHomo sapiens (human)

Compounds (15)

CompoundDescription
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
fumaric acidsee also record for ferrous fumarate; use FUMARATES for general fumaric acid esters
aspartic acidOne of the non-essential amino acids commonly occurring in the L-form. It is found in animals and plants, especially in sugar cane and sugar beets. It may be a neurotransmitter.
Argininosuccinic AcidThis amino acid is formed during the urea cycle from citrulline, aspartate and ATP. This reaction is catalyzed by argininosuccinic acid synthetase.
phosphoglycocyamine
glutamic acid gamma-semialdehydestraight-chain form of delta(1)-pyrroline-5-carboxylate; RN given refers to cpd with unspecified isomeric designation
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).
formic acidRN given refers to parent cpd
delta-1-pyrroline-5-carboxylate(L)-isomer is the biologically active form; RN given refers to cpd without isomeric designation; structure
glycocyamineRN given refers to parent cpd; structure
allophanic acid
N(delta)-acetylornithineRN given refers to (L)-isomer
1-methylhydantoinstructure in first source
n-acetylglutamic acidRN given refers to (L)-isomer