| ALX-801-035-C100 | 100 µg | 271.00 USD | ![]() |
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| Alternative Name: | Bile salt export pump, SPGP, Sister of P-glycoprotein, ABCB11 |
| Concentration: | 1mg/ml |
| Purity Detail: | Protein G-affinity purified. |
| Formulation: | Liquid. Contains 100nM sodium azide and 0.7% BSA. |
| Clone: | USal-hBSEP-McAb-1 |
| Isotype: | Mouse IgG1 |
| Immunogen: | Synthetic peptide corresponding to aa 705-718 (Y705LVHEPPLAVVDHK718) of human BSEP (bile salt export pump). |
| Specificity: | Recognizes human BSEP. Does not cross-react with human P-glycoprotein. |
| Application: | Immunocytochemistry (cytospin preparations) Western Blot (1:50-1:200) |
| Short Term Storage: | +4°C |
| Long Term Storage: | -20°C |
| Handling: | After opening, prepare aliquots and store at -20°C. Avoid freeze/thaw cycles. |
| Miscellaneous/General: | Bile Salts Bile salts are important physiological agents that serve a number of functions, including absorption, solubilization, transport and secretion of lipids. In the liver, they participate in the generation of bile flow and the secretion of cholesterol and phospholipids, such as phosphatidylcholine. When released into the intestine, they facilitate the uptake of cholesterol, fat-soluble vitamins and other lipids. Moreover, the biosynthesis of bile acids from cholesterol is the most significant pathway for the elimination of cholesterol from the body. However, because of their detergent properties, bile acids are inherently cytotoxic and disruptions in their normal transport or secretion can result in a variety of pathophysiological conditions. BSEP 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, the bile salt export pump (BSEP or ABCB11) 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 BSEP gene. PFIC patients with mutations in the BSEP gene have normal γ-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. |

