背景描述Description:
BSEP是ATP结合盒(ABC)转运蛋白的一员,介导胆酸、牛磺胆酸和其他胆酸结合物通过肝细胞小管膜进入小管。BSEP与进行性家族性肝内胆汁淤积症2型(PFIC2)和良性复发性肝内胆汁淤积症2型(BRIC2)有关。BSEP基因突变引起的PFIC2增加了早期的风险。
Bile Salt Export Pump (BSEP) is a member of the ATP-binding cassette (ABC) transporters, which mediates the transport of bile acid, taurocholate and other cholate conjugates across the hepatocyte canalicular membrane into the canaliculus. BSEP is associated with progressive familial intrahepatic cholestasis type 2 (PFIC2) and benign recurrent intrahepatic cholestasis type 2 (BRIC2). PFIC2 caused by mutations in the BSEP gene increases the risk of hepatocellular carcinoma in early life.
Specifications
Clone IHC518
Source Mouse Monoclonal
Positive Control Liver
Dilution Range 1:50-1:200
参考文献References:
Strautnieks S, et al. Nature Genetics. 1998; 20:233–8.
Noé J, et al. Gastroenterology. 2002; 123:1659–66.
Arrese M, et al. Pflügers Archiv. 2004; 449:123–31.
Jansen PL, et al. Gastroenterology. 1999; 117:1370–9.
van Mil SW, et al. Gastroenterology. 2004; 127:379–84.
Knisely AS, et al. Hepatology. 2006; 44:478–86.