Urea cycle

Argininosuccinate synthase or synthetase (ASS; EC 6.3.4.5) is an enzyme that catalyzes the synthesis of argininosuccinate from citrulline and aspartate.

Ornithine transcarbamylase deficiency also known as OTC deficiency is the most common urea cycle disorder in humans.

Urea, also known as carbamide, is an organic compound with the chemical formula CO(NH2)2.

Arginine (abbreviated as Arg or R) encoded by the codons CGU, CGC, CGA, CGG, AGA, and AGG is an α-amino acid that is used in the biosynthesis of proteins.

Aspartic acid (abbreviated as Asp or D; encoded by the codons [GAU and GAC]), also known as aspartate, is an α-amino acid that is used in the biosynthesis of proteins.

Fumaric acid or trans-butenedioic acid is the chemical compound with the formula HO2CCH=CHCO2H.

Ornithine translocase is responsible for transporting ornithine from the cytosol into the mitochondria in the urea cycle.

The organic compound citrulline is an α-amino acid.

Ornithine is a non proteinogenic amino acid that plays a role in the urea cycle.

N-acetylglutamate synthase (NAGS) is an enzyme that catalyses the production of N-Acetylglutamate (NAG) from glutamate and acetyl-CoA.

Arginase (EC 3.5.3.1, arginine amidinase, canavanase, L-arginase, arginine transamidinase) is a manganese-containing enzyme.

Carbamoyl phosphate is an anion of biochemical significance.

Argininosuccinic acid is a chemical compound that is a basic amino acid.

ASL (argininosuccinate lyase, also known as argininosuccinase) is an enzyme that catalyzes the reversible breakdown of argininosuccinate (ASA) producing the amino acid arginine and dicarboxylic acid fumarate.

Carbamoyl Phosphate Synthetase I is a ligase enzyme located in the mitochondria involved in the production of urea.

Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesisof carbamoyl phosphate from glutamine (EC 6.3.5.5) or ammonia (EC 6.3.4.16) and bicarbonate.