本公司经销ETBR/Endothelin B Receptor，内皮素B受体抗体，克隆类型为polyclonal，宿主来源是Rabbit，ETBR/Endothelin B Receptor内皮素B受体抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-4198R
英文名称：Anti-ETBR/Endothelin B Receptor
中文名称：内皮素B受体抗体
其他名称：名ABCDS; Ednra; Ednrb; EDNRB_HUMAN; Endothelin B receptor; Endothelin B receptor precursor; Endothelin receptor Non selective type; Endothelin receptor non-selective type; Endothelin receptor type B; ET B; ET-B; ET-BR; ETB; ETRB; Hirschsprung disease 2; HSCR; HSCR2; EDNRB; HSCR; WS4A.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 47kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, pig, dog, Rb, cow, hrs, chk
产品介绍：The protein encoded by this gene is a G protein-coupled receptor which activates a phosphatidylinositol-calcium second messenger system. Its ligand, endothelin, consists of a family of three potent vasoactive peptides: ET1, ET2, ａnd ET3. Studies suggest that the multigenic disorder, Hirschsprung disease type 2, is due to mutations in the endothelin receptor type B gene. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq].Function : Non-specific receptor for endothelin 1, 2, ａnd 3. Mediates its action by association with G proteins that activate a phosphatidylinositol-calcium second messenger system.Subcellular Location : Cell membrane; Multi-pass membrane protein.Tissue Specificity : Expressed in placental stem villi vessels, but not in cultured placental villi smooth muscle cells.Post-translational modifications : Palmitoylation of Cys-402 was confirmed by the palmitoylation of Cys-402 in a deletion mutant lacking both Cys-403 ａnd Cys-405.DISEASE : Defects in EDNRB are a cause of Waardenburg syndrome type 4A （WS4A） [MIM:277580]; also known as Waardenburg-Shah syndrome. WS4A is characterized by the association of Waardenburg features （depigmentation ａnd deafness） ａnd the absence of enteric ganglia in the distal part of the intestine （Hirschsprung disease）.Defects in EDNRB are the cause of Hirschsprung disease type 2 （HSCR2） [MIM:600155]; also known as aganglionic megacolon （MGC）. HSCR2 is a congenital disorder characterized by absence of enteric ganglia along a variable length of the intestine. It is the most common cause of congenital intestinal obstruction. Early symptoms range from complete acute neonatal obstruction, characterized by vomiting, abdominal distention ａnd failure to pass stool, to chronic constipation in the older child.Defects in EDNRB are the cause of ABCD syndrome （ABCDS） [MIM:600501]. ABCD syndrome is an autosomal recessive syndrome characterized by albinism, black lock at temporal occipital region, bilateral deafness, aganglionosis of the large intestine ａnd total absence of neurocytes ａnd nerve fibers in the small intestine.Similarity : Belongs to the G-protein coupled receptor 1 family. Endothelin receptor subfamily. EDNRB sub-subfamily.产品图片Overlay histogram showing SP2/0 cells stained with bs-4198R-PE （red line）. The cells were fixed with 1%paraformaldehyde （10 min） ａnd then permeabilized with 1x PBS /0.2%TritonX-100 for 5 min. The cells were then incubated in 1x PBS / 1%BSA to block non-specific protein-protein interactions. The cells were then incubated with the antibody （bs-4198R-PE, 2ug/1x106 cells） for 30 min at 22-25°C. Isotype control antibody （black line） was rabbit IgG （2ug/1x106 cells） used under the same conditions. Acquisition of >5,000 events was performed.