Target：ECE1

Gene Name：ECE1

Protein Name：Endothelin-converting enzyme 1 (ECE-1) (EC 3.4.24.71)

Human Gene Id：1889

Human Swiss Prot No：P42892

Mouse Swiss Prot No：Q4PZA2

Rat Swiss Prot No：P42893

Immunogen：Synthesized peptide derived from part region of human protein

Specificity：ECE1 Polyclonal Antibody detects endogenous levels of protein.

Formulation：Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500-2000 ELISA 1:5000-20000

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD)：84kD

Background： The protein encoded by this gene is involved in proteolytic processing of endothelin precursors to biologically active peptides. Mutations in this gene are associated with Hirschsprung disease, cardiac defects and autonomic dysfunction. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene.[provided by RefSeq, Sep 2009],

Function：catalytic activity:Hydrolysis of the 21-Trp-|-Val-22 bond in big endothelin to form endothelin 1.,cofactor:Binds 1 zinc ion per subunit.,disease:Defects in ECE1 are a cause of Hirschsprung disease, cardiac defects and autonomic dysfunction [MIM:600423]. It is a form of Hirschsprung disease [MIM:142623] with skip-lesions defects, craniofacial abnormalities and other dysmorphic features, and autonomic dysfunction.,enzyme regulation:Inhibited by phosphoramidon.,function:Converts big endothelin-1 to endothelin-1.,similarity:Belongs to the peptidase M13 family.,subunit:Homodimer; disulfide-linked.,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 s

Subcellular Location：Cell membrane; Single-pass type II membrane protein.

Expression：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.