Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1

[1] Bull LN(1998)A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis Nat Genet 18 219-224

[2] van Eijk MJ(2011)Progressive familial intrahepatic cholestasis (PFIC) type 1, 2, and 3: a review of the liver pathology findings Semin Liver Dis 31 3-10

[3] Pawlikowska L(2011)Progressive familial intrahepatic cholestasis: a single-center experience of living-donor liver transplantation during two decades in Japan Clin Transplant 25 776-785

[4] DeYoung JA(2008)ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity Hepatology 47 268-278

[5] Juijn JA(2006)Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport Hepatology 44 195-204

[6] Liao M(2002)The human bile salt export pump: characterization of substrate specificity and identification of inhibitors Gastroenterology 123 1649-1658

[7] Klomp LW(1998)The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver J Biol Chem 273 10046-10050

[8] Lomri N(2005)Transport by vesicles of glycine- and taurine-conjugated bile salts and taurolithocholate 3-sulfate: a comparison of human BSEP with rat Bsep Biochim Biophys Acta 1738 54-62

[9] Berger R(2002)Functional expression of the canalicular bile salt export pump of human liver Gastroenterology 123 1659-1666

[10] Scharschmidt BF(2009)Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol content J Biol Chem 284 9947-9954

[1] Bull LN(1998)A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis Nat Genet 18 219-224

[2] van Eijk MJ(2011)Progressive familial intrahepatic cholestasis (PFIC) type 1, 2, and 3: a review of the liver pathology findings Semin Liver Dis 31 3-10

[3] Pawlikowska L(2011)Progressive familial intrahepatic cholestasis: a single-center experience of living-donor liver transplantation during two decades in Japan Clin Transplant 25 776-785

[4] DeYoung JA(2008)ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity Hepatology 47 268-278

[5] Juijn JA(2006)Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport Hepatology 44 195-204

[6] Liao M(2002)The human bile salt export pump: characterization of substrate specificity and identification of inhibitors Gastroenterology 123 1649-1658

[7] Klomp LW(1998)The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver J Biol Chem 273 10046-10050

[8] Lomri N(2005)Transport by vesicles of glycine- and taurine-conjugated bile salts and taurolithocholate 3-sulfate: a comparison of human BSEP with rat Bsep Biochim Biophys Acta 1738 54-62

[9] Berger R(2002)Functional expression of the canalicular bile salt export pump of human liver Gastroenterology 123 1659-1666

[10] Scharschmidt BF(2009)Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol content J Biol Chem 284 9947-9954