本公司经销BSEP/ABCB11，胆汁酸盐输出泵蛋白抗体，克隆类型为polyclonal，宿主来源是Rabbit，BSEP/ABCB11胆汁酸盐输出泵蛋白抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-1954R
英文名称：Anti-BSEP/ABCB11
中文名称：胆汁酸盐输出泵蛋白抗体
其他名称：Bile salt export pump; ATP binding cassette sub family B （MDR/TAP） member 11; ATP binding cassette sub family B member 11; Bsep; Sister of P glycoprotein; Spgp; Abcb11; ABC16; Lith1; PFIC2; PGY4.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 145kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, Rb
产品介绍：The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette （ABC） transporters. ABC proteins transport various molecules across extra- ａnd intra-cellular membranes. ABC genes are divided into seven distinct subfamilies （ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White）. This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt transporter in humans ａnd mice. Mutations in the human gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy. Bile formation is an important function of the liver. It is mediated by hepatocytes which generate bile flow within the bile canaliculi by continuous vectorial secretion of bile salts ａnd other solutes across their canalicular （apical） membrane. Bile secretion is mediated by several ATP-binding cassette （ABC） transporters located in the canalicular membrane of hepatocytes. Among these ABC transporters, ABCB11 （bile salt export pump or BSEP） represents the primary, if not sole transport system for the canalicular excretion of bile salts. Bile secretory failure results in cholestasis ａnd progressive familial intrahepatic cholestasis （PFIC） in infancy represents a group of inherited cholestatic diseases that are classified into three subtypes. One of these subtypes, PFIC II, is associated with mutations in the ABCB11 gene. PFIC patients with mutations in the ABCB11 gene have normal serum gamma-glutamyltransferase activity, low concentrations of bile salts in bile, ａnd an absence of bile duct proliferation. Additionally, human obesity is associated with altered cholesterol homeostasis including increased production ａnd turnover, as well as secretion of excess cholesterol from the liver into bile. ABCB11 is a multifunctional polypeptide with two homologous halves, each containing a hydrophobic membrane ancoring domain ａnd an ATP binding cassette （ABC） domain. The membrane anchoring domain is composed of six helixes buried in the lipid bilayer of the plasma membrane ａnd the ATP binding ABCs are exposed to the cytosol. The membrane anchoring domain helixes are thought to form channels spanning the plasma membrane.Subunit : Interacts with HAX1 （By similarity）.Subcellular Location : Membrane; Multi-pass membrane protein.Tissue Specificity : Expressed predominantly, if not exclusively in the liver, where it was further localized to the canalicular microvilli ａnd to subcanalicular vesicles of the hepatocytes by in situ （By similarity）.Similarity : Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter （TC 3.A.1.201） subfamily. Contains 2 ABC transmembrane type-1 domains. Contains 2 ABC transporter domains.