Ornithine transcarbamylase

OTC
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1OTH, 1C9Y, 1EP9, 1FVO
Identifiers
Aliases	OTC, OCTD, ornithine carbamoyltransferase, ornithine transcarbamylase, OTCD
External IDs	OMIM: 300461; MGI: 97448; HomoloGene: 446; GeneCards: OTC; OMA:OTC - orthologs
Gene location (Human)
Chr.	X chromosome (human)
End	38,421,446 bp
Gene location (Mouse)
Chr.	X chromosome (mouse)
End	10,187,263 bp
RNA expression pattern
	Top expressed in
	jejunal mucosa; ; right lobe of liver; ; duodenum; ; mucosa of transverse colon; ; mucosa of ileum; ; testicle; ; rectum; ; gonad; ; right coronary artery; ; pancreatic ductal cell;
	Top expressed in
	left lobe of liver; ; Ileal epithelium; ; gallbladder; ; jejunum; ; migratory enteric neural crest cell; ; Paneth cell; ; duodenum; ; crypt of lieberkuhn of small intestine; ; human fetus; ; fetal liver hematopoietic progenitor cell;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	amino acid binding; transferase activity; carboxyl- or carbamoyltransferase activity; phosphate ion binding; phospholipid binding; ornithine carbamoyltransferase activity;
Cellular component	cytoplasm; mitochondrial inner membrane; mitochondrial matrix; mitochondrion;
Biological process	response to zinc ion; cellular amino acid biosynthetic process; ornithine catabolic process; midgut development; cellular amino acid metabolic process; response to nutrient levels; response to biotin; anion homeostasis; liver development; arginine biosynthetic process; response to insulin; ammonium homeostasis; ornithine metabolic process; protein homotrimerization; urea cycle; citrulline biosynthetic process; arginine biosynthetic process via ornithine;
	Sources:Amigo / QuickGO
Orthologs
	5009
	18416
	ENSG00000036473
	ENSMUSG00000031173
	P00480
	P11725
	NM_000531
	NM_008769
	NP_000522
	NP_032795
	Wikidata
View/Edit Human	View/Edit Mouse

Ornithine transcarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme (EC 2.1.3.3) that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) to form citrulline (Cit) and phosphate (P_i). There are two classes of OTC: anabolic and catabolic. This article focuses on anabolic OTC. Anabolic OTC facilitates the sixth step in the biosynthesis of the amino acid arginine in prokaryotes. In contrast, mammalian OTC plays an essential role in the urea cycle, the purpose of which is to capture toxic ammonia and transform it into urea, a less toxic nitrogen source, for excretion.