ECE1 (endothelin converting enzyme 1) is a zinc-dependent endopeptidase that catalyzes the conversion of inactive big endothelin-1 to the active 21-amino acid peptide endothelin-1 1. This enzyme exists in four isoforms (ECE-1a-d) derived from alternative promoters of a single gene 1. ECE1 is localized to multiple cellular compartments including the external plasma membrane, endosomes, lysosomes, and Weibel-Palade bodies—specialized secretory granules in vascular endothelial cells 1. Endothelin-1 is one of the most potent vasoconstrictors known and is released constitutively to maintain vascular tone and in response to external stimuli 1. ECE1 dysfunction is clinically significant in multiple contexts. Deletions encompassing ECE1 on chromosome 1 contribute to 1p36 deletion syndrome, characterized by developmental delay, intellectual disability, congenital heart defects, cardiomyopathy, and other anomalies 2. ECE1 gene polymorphisms show gender-specific associations with essential hypertension susceptibility in Han Chinese populations, with certain variants influencing blood pressure regulation 3. Additionally, ECE1 encodes the extent of cell elongation 1 protein, which produces candidalysin—a fungal toxin that exacerbates alcohol-associated liver disease and directly damages hepatocytes 4. These findings establish ECE1 as a multifunctional gene affecting endocrine signaling, cardiovascular development, blood pressure homeostasis, and host-pathogen interactions.