SPGP

S. J., Helgason, C. D., Ackerley, C., Phillips, M. J., Ling, V., Targeted inactivation of sister of P-glycoprotein gene (spgp) in mice results in nonprogressive but persistent intrahepatic cholestasis, Proc. Natl. Acad. Sci. USA 2001, 98, 2011-2016. 

BSEP also known as sister-P-glycoprotein (SPGP) was originally cloned from pig liver (185). BSEP is localized on the canalicular membrane of hepa-tocytes and is responsible for the secretion of bile salts across the canalicular membrane into bile. BSEP appears to be the predominant bile salt efflux system for hepatocytes, and is a critical component in the enterohepatic circulation of bile acids. A number of mutations in the transporter were found to the basis for progressive familial intrahepatic cholestasis type 2 (PFIC2) (186-188). Mutations found in PFIC2 patients include frameshifts, missense mutations, and premature termination codons. Most PFIC2 patients lack immunohistochemically detectable BSEP in their liver. Recently, seven... 

BSEPf ABCB11 SPGP Liver canalicular membrane Apical PFIC2... 

BSEP, SPGP ABCB11 Liver Bile salts Apical 27,55,182... 

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