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)[1] Band Xp11.4 Start 38,352,586 bp[1] End 38,421,446 bp[1]
Gene location (Mouse) Chr. X chromosome (mouse)[2] Band X A1.1|X 4.66 cM Start 10,118,544 bp[2] End 10,187,263 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) 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 BioGPS 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 Species Human Mouse Entrez 5009 18416 Ensembl ENSG00000036473 ENSMUSG00000031173 UniProt P00480 P11725 RefSeq (mRNA) NM_000531 NM_008769 RefSeq (protein) NP_000522 NP_032795 Location (UCSC) Chr X: 38.35 – 38.42 Mb Chr X: 10.12 – 10.19 Mb PubMed search [3] [4]
Wikidata
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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.^[5] 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.