ECE1 / ECE-1 抗体

ECE1 / ECE-1 抗体产品信息免疫原 : Recombinant Human ECE1 / ECE-1 protein (Catalog#10887-H07H)

Antibody Type : Rabbit Polyclonal Antibody ( Antibody Purification Platform )
抗体宿主 : Rabbit IgG

缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 : Produced in rabbits immunized with purified, recombinant Human ECE1 / ECE-1 (rh ECE1 / ECE-1; Catalog#10887-H07H; P42892-1; Gln 90-Trp 770). ECE1 / ECE-1 specific IgG was purified by Human ECE1 / ECE-1 affinity chromatography.
ECE1 / ECE-1 抗体 Background
Endothelin-converting enzyme 1, also known as ECE-1, is a single-pass type II membrane protein which belongs to thepeptidase M13 family. ECE-1 converts big endothelin-1 to endothelin-1. ECE-1 is a membrane metalloprotease that generates endothelin from its direct precursor big endothelin. Four isoforms of ECE-1 are produced from a single gene through the use of alternate promoters. These isoforms share the same extracellular catalytic domain and contain unique cytosolic tails, which results in their specific subcellular targeting.All isoforms of ECE-1 are expressed in umbilical vein endothelial cells, polynuclear neutrophils, fibroblasts, atrium cardiomyocytes and ventricles. Isoforms A, B and C of ECE-1 are also expressed in placenta, lung, heart, adrenal gland and phaeochromocytoma; isoforms A and C of ECE-1 in liver, testis and small intestine; isoform B, C and D of ECE-1 in endothelial cells and umbilical vein smooth muscle cells; isoforms C and D in saphenous vein cells, and isoform C in kidney. Defects in ECE1 are a cause of Hirschsprung disease, cardiac defects and autonomic dysfunction. It is a form of Hirschsprung disease with skip-lesions defects, craniofacial abnormalities and other dysmorphic features, and autonomic dysfunction.
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