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endothelin converting enzyme-1; ECE-1 (ECE1)
Function:
- converts big endothelin-1 to endothelin-1
- inhibited by phosphoramidon
- hydrolysis of 21-Trp-|-Val-22 bond in big endothelin to form endothelin 1
- substrates:
a) big endothelins
b) bradykinin
c) substance P
d) oxidized insulin B chain
e) amyloid A4 peptide [4]
- inhibitors: phosphoramidon
Cofactor: binds 1 Zn+2 per subunit (putative)
Structure:
- homodimer
- belongs to the peptidase M13 family
Compartment: membrane, plasma membrane, internal membranes
Alternative splicing: named isoforms=4, A-D
Expression:
- all isoforms are expressed in umbilical vein endothelial cells, polynuclear neutrophils, fibroblasts, atrium cardiomyocytes & ventricles
- isoforms A, B & C are also expressed in placenta, lung, heart, adrenal gland & pheochromocytoma
- isoforms A & C in liver, testis & small intestine
- isoform B, C & D in endothelial cells & umbilical vein smooth muscle cells
- isoforms C & D in saphenous vein cells, & isoform C in kidney
Pathology:
- defects in ECE1 are a cause of Hirschsprung disease, cardiac defects & autonomic dysfunction
General
endothelin-converting enzyme
glycoprotein
phosphoprotein
Properties
SIZE: entity length = 770 aa
MW = 87 kD
COMPARTMENT: cellular membrane
MOTIF: Ser phosphorylation site {S34}
transmembrane domain {69-89}
N-glycosylation site {N166}
N-glycosylation site {N187}
N-glycosylation site {N210}
N-glycosylation site {N270}
N-glycosylation site {N316}
N-glycosylation site {N362}
N-glycosylation site {N383}
N-glycosylation site {N539}
Zn+2-binding site
SITE: 607-607
glutamate residue {E608}
Zn+2-binding site
SITE: 611-611
N-glycosylation site {N632}
N-glycosylation site {N651}
Zn+2-binding site
SITE: 667-667
aspartate residue {D671}
Database Correlations
OMIM correlations
MORBIDMAP 600423
UniProt P42892
PFAM correlations
Entrez Gene 1889
Kegg hsa:1889
ENZYME 3.4.24.71
References
- UniProt :accession P42892
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=ECE1
- NIEHS-SNPs
http://egp.gs.washington.edu/data/ece1/
- Selkoe DJ.
Clearing the brain's amyloid cobwebs.
Neuron. 2001 Oct 25;32(2):177-80. Review.
PMID: 11683988