产品名称
ECE1 Rabbit Polyclonal Antibody
蛋白名称
Endothelin-converting enzyme 1 (ECE-1) (EC 3.4.24.71)
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
P42892
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P42892/entry
Mouse Swissprot No.
Q4PZA2
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q4PZA2
Rat Swissprot Link
http://www.uniprot.org/uniprot/O54941P42893
免疫原
Synthesized peptide derived from part region of human protein
特异性
ECE1 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
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.
细胞定位
Cell membrane; Single-pass type II membrane protein.
功能
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 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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
