Ornithine Carbamoyltransferase
A urea cycle enzyme that catalyzes the formation of orthophosphate and L-citrulline (CITRULLINE) from CARBAMOYL PHOSPHATE and L-ornithine (ORNITHINE). Deficiency of this enzyme may be transmitted as an X-linked trait. EC 2.1.3.3.
Ornithine Carbamoyltransferase Deficiency Disease
An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)
Carboxyl and Carbamoyl Transferases
Carbamyl Phosphate
The 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).
Carbamoyl-Phosphate Synthase (Ammonia)
Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing)
An enzyme that catalyzes the formation of carbamoyl phosphate from ATP, carbon dioxide, and glutamine. This enzyme is important in the de novo biosynthesis of pyrimidines. EC 6.3.5.5.
Transferases
Transferases are enzymes transferring a group, for example, the methyl group or a glycosyl group, from one compound (generally regarded as donor) to another compound (generally regarded as acceptor). The classification is based on the scheme "donor:acceptor group transferase". (Enzyme Nomenclature, 1992) EC 2.
Aspartate Carbamoyltransferase
Carbamates
Derivatives of carbamic acid, H2NC(=O)OH. Included under this heading are N-substituted and O-substituted carbamic acids. In general carbamate esters are referred to as urethanes, and polymers that include repeating units of carbamate are referred to as POLYURETHANES. Note however that polyurethanes are derived from the polymerization of ISOCYANATES and the singular term URETHANE refers to the ethyl ester of carbamic acid.
Glutathione Transferase
Alkyl and Aryl Transferases
Phosphotransferases (Carboxyl Group Acceptor)
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Transferases (Other Substituted Phosphate Groups)
Carbon-Nitrogen Ligases
Amino Acid Sequence
Ammonia
A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. Note that the aqueous form of ammonia is referred to as AMMONIUM HYDROXIDE.
Citrulline
Citrulline is an α-amino acid, primarily produced in the urea cycle in the liver and found in some dietary proteins, which functions as a vital intermediator in the nitrogen metabolism and vasodilation, and can be supplemented for potential health benefits in improving blood flow, reducing fatigue, and enhancing exercise performance.
Orotic Acid
Orotic acid, also known as pyrophosphoric acid dihydrate, is a organic compound that plays a role in the biosynthesis of pyrimidines, and elevated levels of orotic acid in urine can indicate certain genetic disorders or liver dysfunction.
DNA Nucleotidylexotransferase
Escherichia coli
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
Azauridine
Coenzyme A-Transferases
Ligases
Dogfish
Sharks of the family Squalidae, also called dogfish sharks. They comprise at least eight genera and 44 species. Their LIVER is valued for its oil and its flesh is often made into fertilizer.
Binding Sites
Substrate Specificity
Phosphonoacetic Acid
Glutamine
A non-essential amino acid present abundantly throughout the body and is involved in many metabolic processes. It is synthesized from GLUTAMIC ACID and AMMONIA. It is the principal carrier of NITROGEN in the body and is an important energy source for many cells.
Dihydroorotase
Uridine Monophosphate
Aspartic Acid
Urea
A 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.