Targeted inactivation of sister of P-glycoprotein gene (spgp) in mice results in nonprogressive but persistent intrahepatic cholestasis.

@article{Wang2001TargetedIO,
  title={Targeted inactivation of sister of P-glycoprotein gene (spgp) in mice results in nonprogressive but persistent intrahepatic cholestasis.},
  author={Renxue Wang and Mohamed Abouelmagd Salem and Ibrahim M. Yousef and Beatriz Tuchweber and Ping Hai Lam and Sarah J. Childs and Cheryl D. Helgason and Cameron A. Ackerley and Melville J Phillips and Victor Ling},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2001},
  volume={98 4},
  pages={2011-6}
}
Mutations in the sister of P-glycoprotein (Spgp) or bile salt export pump (BSEP) are associated with Progressive Familial Intrahepatic Cholestasis (PFIC2). Spgp is predominantly expressed in the canalicular membranes of liver. Consistent with in vitro evidence demonstrating the involvement of Spgp in bile salt transport, PFIC2 patients secrete less than 1% of biliary bile salts compared with normal infants. The disease rapidly progresses to hepatic failure requiring liver transplantation before… CONTINUE READING
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