Function: Converts big endothelin-1 to endothelin-1.
Tissue specificity: All isoforms are expressed in umbilical vein endothelial cells, polynuclear neutrophils, fibroblasts, atrium cardiomyocytes and ventricles. Isoforms A, B and C are also expressed in placenta, lung, heart, adrenal gland and phaeochromocytoma; isoforms A and C in liver, testis and small intestine; isoform B, C and D in endothelial cells and umbilical vein smooth muscle cells; isoforms C and D in saphenous vein cells, and isoform C in kidney.
Disease: Hirschsprung disease cardiac defects and autonomic dysfunction
Similarity: Belongs to the peptidase M13 family.
Cell Membrane. Single pass type II membrane protein.
Mous DS et al. Changes in vasoactive pathways in congenital diaphragmatic hernia associated pulmonary hypertension explain unresponsiveness to pharmacotherapy. Respir Res18:187 (2017).
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