Endothelin-converting enzyme (IPR029733)

Short name: ECE1/ECE2

Overlapping homologous superfamilies

Family relationships

  • Peptidase M13 (IPR000718)
    • Endothelin-converting enzyme (IPR029733)


Endothelin-converting enzyme 1 (ECE1) is an enzyme that converts big endothelin-1 (big-ET-1) to endothelin-1 (ET-1). ET-1 is a highly potent vasoconstrictor that regulates vascular tone and blood pressure [PMID: 22728136]. ECE-1 gene encodes four different isoforms of ECE-1 (ECE-1a, 1b, 1c and 1d), each one under the control of different promoters [PMID: 22728136]. The localisation of ECE-1 at the cell surface can be regulated through phosphorylation by protein kinase C [PMID: 19617920]. It can be post-transcriptionally regulated by alternative polyadenylation (APA) [PMID: 24497914]. It is capable of cleaving amyloid-beta (Abeta) and hence linked to Alzheimer's disease [PMID: 23629587, PMID: 22330820]. ECE-1 gene expression is upregulated in a number of cancers, including prostate cancer [PMID: 24497914].

Endothelin-converting enzyme 2 (ECE2) is a non-classical neuropeptide processing enzyme that processes various neuroendocrine peptides, including neurotensin, angiotensin I, substance P, proenkephalin-derived peptides, and prodynorphin-derived peptides [PMID: 21972895, PMID: 12560336]. ECE2 was named for its ability of generating the potent vasoconstrictor endothelin-1 (ET-1) from its precursor big-ET-1 [PMID: 7797512]. ECE-2 KO mice show increased amyloid beta-protein accumulation in the brain and exhibit abnormal responses to morphine and altered peptide levels in the spinal cord [PMID: 12464614, PMID: 21972895].

This entry also includes Drosophila Neprilysin-3, which is a thermolysin-like zinc metalloendopeptidase, plays an important role in turning off peptide signalling events at the cell surface [PMID: 11223883].

GO terms

Biological Process

GO:0016486 peptide hormone processing

Molecular Function

GO:0004222 metalloendopeptidase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.