// Workers AI · dad joke modeWhat did endothelin converting enzyme 1 say? I'm converting.

ECE1
Identifiers
Aliases	ECE1, ECE, endothelin converting enzyme 1
External IDs	OMIM: 600423; MGI: 1101357; HomoloGene: 1068; GeneCards: ECE1; OMA:ECE1 - orthologs
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 3DWB
Enzyme activity EC # BRENDA ExPASy KEGG MetaCyc 3.4.24.71 ↗ ↗ ↗ ↗
Gene location (Human) Chr. Chromosome 1 (human) Band 1p36.12 Start 21,217,247 bp End 21,345,572 bp
Gene location (Mouse) Chr. Chromosome 4 (mouse) Band 4|4 D3 Start 137,589,548 bp End 137,692,540 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in stromal cell of endometrium left adrenal cortex right adrenal gland popliteal artery tibial arteries right adrenal cortex right lung right coronary artery gastric mucosa canal of the cervix Top expressed in aortic valve right lung lobe endothelial cell of lymphatic vessel ascending aorta islet of Langerhans gastrula lip pituitary gland internal carotid artery external carotid artery More reference expression data BioGPS More reference expression data
Gene ontology Molecular function endopeptidase activity metal ion binding peptide hormone binding peptidase activity protein binding hydrolase activity metallopeptidase activity protein homodimerization activity metalloendopeptidase activity zinc ion binding Cellular component Weibel-Palade body integral component of membrane vesicle endosome membrane lysosomal membrane intrinsic component of endosome membrane perinuclear region of cytoplasm extracellular exosome external side of plasma membrane early endosome plasma membrane Biological process endothelin maturation bradykinin catabolic process regulation of systemic arterial blood pressure by endothelin embryonic digit morphogenesis ear development hormone catabolic process proteolysis development of the heart calcitonin catabolic process regulation of vasoconstriction substance P catabolic process pharyngeal system development positive regulation of receptor recycling protein processing peptide hormone processing Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 1889 230857 Ensembl ENSG00000117298 ENSMUSG00000057530 UniProt P42892 Q4PZA2 RefSeq (mRNA) NM_001113347 NM_001113348 NM_001113349 NM_001397 NM_199307 NM_001369177 NM_001369178 NM_001369179 RefSeq (protein) NP_001106818 NP_001106819 NP_001106820 NP_001388 NP_001106820.1 NP_955011 NP_001356106 NP_001356107 NP_001356108 Location (UCSC) Chr 1: 21.22 – 21.35 Mb Chr 4: 137.59 – 137.69 Mb PubMed search
Wikidata
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Endothelin converting enzyme 1, also known as ECE1, is an enzyme which in humans is encoded by the ECE1 gene. It is inhibited by phosphoramidon. ECE1 also mediates melanin production through breaking down inactive Big-EDN1 to produce an active EDN1 in keratinocytes and endothelial cells.

Function

Endothelin-converting enzyme-1 is involved in the proteolytic processing of endothelin-1 (EDN1), -2 (EDN2), and -3 (EDN3) to biologically active peptides.

Further reading

• Yorimitsu K, Moroi K, Inagaki N, et al. (1995). "Cloning and sequencing of a human endothelin converting enzyme in renal adenocarcinoma (ACHN) cells producing endothelin-2". Biochem. Biophys. Res. Commun. 208 (2): 721–7. doi:10.1006/bbrc.1995.1397. PMID 7695628.
• Schmidt M, Kröger B, Jacob E, et al. (1995). "Molecular characterization of human and bovine endothelin converting enzyme (ECE-1)". FEBS Lett. 356 (2–3): 238–43. doi:10.1016/0014-5793(94)01277-6. PMID 7805846.
• Shimada K, Matsushita Y, Wakabayashi K, et al. (1995). "Cloning and functional expression of human endothelin-converting enzyme cDNA". Biochem. Biophys. Res. Commun. 207 (2): 807–12. doi:10.1006/bbrc.1995.1258. PMID 7864876.
• Valdenaire O, Rohrbacher E, Mattei MG (1996). "Organization of the gene encoding the human endothelin-converting enzyme (ECE-1)". J. Biol. Chem. 270 (50): 29794–8. doi:10.1074/jbc.270.50.29794. PMID 8530372.
• Matsuoka R, Sawamura T, Yamada K, et al. (1996). "Human endothelin converting enzyme gene (ECE1) mapped to chromosomal region 1p36.1". Cytogenet. Cell Genet. 72 (4): 322–4. doi:10.1159/000134214. PMID 8641140.
• Orzechowski HD, Richter CM, Funke-Kaiser H, et al. (1997). "Evidence of alternative promoters directing isoform-specific expression of human endothelin-converting enzyme-1 mRNA in cultured endothelial cells". J. Mol. Med. 75 (7): 512–21. doi:10.1007/s001090050136. PMID 9253714. S2CID 34633812.
• Schweizer A, Valdenaire O, Nelböck P, et al. (1998). "Human endothelin-converting enzyme (ECE-1): three isoforms with distinct subcellular localizations". Biochem. J. 328 (Pt 3): 871–7. doi:10.1042/bj3280871. PMC 1218999. PMID 9396733.
• Kido T, Sawamura T, Masaki T (1998). "The processing pathway of endothelin-1 production". J. Cardiovasc. Pharmacol. 31 (Suppl 1): S13–5. doi:10.1097/00005344-199800001-00006. PMID 9595387.
• Hofstra RM, Valdenaire O, Arch E, et al. (1999). "A loss-of-function mutation in the endothelin-converting enzyme 1 (ECE-1) associated with Hirschsprung disease, cardiac defects, and autonomic dysfunction". Am. J. Hum. Genet. 64 (1): 304–8. doi:10.1086/302184. PMC 1377732. PMID 9915973.
• Lee S, Lin M, Mele A, et al. (1999). "Proteolytic processing of big endothelin-3 by the kell blood group protein". Blood. 94 (4): 1440–50. doi:10.1182/blood.V94.4.1440. PMID 10438732.
• Valdenaire O, Lepailleur-Enouf D, Egidy G, et al. (1999). "A fourth isoform of endothelin-converting enzyme (ECE-1) is generated from an additional promoter molecular cloning and characterization". Eur. J. Biochem. 264 (2): 341–9. doi:10.1046/j.1432-1327.1999.00602.x. PMID 10491078.
• Fahnoe DC, Johnson GD, Herman SB, Ahn K (2000). "Disulfide bonds in big ET-1 are essential for the specific cleavage at the Trp(21)-Val(22) bond by soluble endothelin converting enzyme-1 from baculovirus/insect cells". Arch. Biochem. Biophys. 373 (2): 385–93. doi:10.1006/abbi.1999.1586. PMID 10620363.
• López-Ongil S, Senchak V, Saura M, et al. (2000). "Superoxide regulation of endothelin-converting enzyme". J. Biol. Chem. 275 (34): 26423–7. doi:10.1074/jbc.M000767200. PMID 10833511.
• Hachiya A, Kobayashi T, Takema Y, Imokawa G (2002). "Biochemical characterization of endothelin-converting enzyme-1alpha in cultured skin-derived cells and its postulated role in the stimulation of melanogenesis in human epidermis". J. Biol. Chem. 277 (7): 5395–403. doi:10.1074/jbc.M105874200. PMID 11723113.
• Inguimbert N, Coric P, Poras H, et al. (2002). "Toward an optimal joint recognition of the S1' subsites of endothelin converting enzyme-1 (ECE-1), angiotensin converting enzyme (ACE), and neutral endopeptidase (NEP)". J. Med. Chem. 45 (7): 1477–86. doi:10.1021/jm0005454. PMID 11906289.
• MacLeod KJ, Husain RD, Gage DA, Ahn K (2003). "Constitutive phosphorylation of human endothelin-converting enzyme-1 isoforms". J. Biol. Chem. 277 (48): 46355–63. doi:10.1074/jbc.M207972200. PMID 12244060.
• Muller L, Barret A, Etienne E, et al. (2003). "Heterodimerization of endothelin-converting enzyme-1 isoforms regulates the subcellular distribution of this metalloprotease". J. Biol. Chem. 278 (1): 545–55. doi:10.1074/jbc.M208949200. PMID 12393864.
• Eckman EA, Watson M, Marlow L, et al. (2003). "Alzheimer's disease beta-amyloid peptide is increased in mice deficient in endothelin-converting enzyme". J. Biol. Chem. 278 (4): 2081–4. doi:10.1074/jbc.C200642200. PMID 12464614.
• Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. Bibcode:2002PNAS...9916899M. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
• Masatsugu K, Itoh H, Chun TH, et al. (2003). "Shear stress attenuates endothelin and endothelin-converting enzyme expression through oxidative stress". Regul. Pept. 111 (1–3): 13–9. doi:10.1016/S0167-0115(02)00219-7. hdl:2433/147555. PMID 12609744. S2CID 37039969.
• Smollich M, Götte M, Yip GW, et al. (2007). "On the role of endothelin-converting enzyme 1 (ECE-1) and neprilysin in human breast cancer". Breast Cancer Res. Treat. 106 (3): 361–9. doi:10.1007/s10549-007-9516-9. PMID 17295044. S2CID 5546294.