• 1
    Geier A, Wagner M, Dietrich CG, Trauner M. Principles of hepatic organic anion transporter regulation during cholestasis, inflammation and liver regeneration. Biochim Biophys Acta 2007; 1773: 283308.
  • 2
    Gerloff T, Stieger B, Hagenbuch B, Madon J, Landmann L, Roth J, et al. The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver. J Biol Chem 1998; 273: 1004610050.
  • 3
    Stieger B, Meier Y, Meier PJ. The bile salt export pump. Pflugers Arch 2007; 453: 611620.
  • 4
    Lang C, Meier Y, Stieger B, Beuers U, Lang T, Kerb R, et al. Mutations and polymorphisms in the bile salt export pump and the multidrug resistance protein 3 associated with drug-induced liver injury. Pharmacogenet Genomics 2007; 17: 4760.
  • 5
    Wang L, Soroka CJ, Boyer JL. The role of bile salt export pump mutations in progressive familial intrahepatic cholestasis type II. J Clin Invest 2002; 110: 965972.
  • 6
    Plass JR, Mol O, Heegsma J, Geuken M, de Bruin J, Elling G, et al. A progressive familial intrahepatic cholestasis type 2 mutation causes an unstable, temperature-sensitive bile salt export pump. J Hepatol 2004; 40: 2430.
  • 7
    Rubenstein RC, Egan ME, Zeitlin PL. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. J Clin Invest 1997; 100: 24572465.
  • 8
    Hayashi H, Takada T, Suzuki H, Akita H, Sugiyama Y. Two common PFIC2 mutations are associated with the impaired membrane trafficking of BSEP/ABCB11. HEPATOLOGY 2005; 41: 916924.
  • 9
    Li C, Ramjeesingh M, Reyes E, Jensen T, Chang X, Rommens JM, et al. The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR. Nat Genet 1993; 3: 311316.
  • 10
    Rubenstein RC, Zeitlin PL. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med 1998; 157: 484490.
  • 11
    Singh OV, Vij N, Mogayzel PJ Jr, Jozwik C, Pollard HB, Zeitlin PL. Pharmacoproteomics of 4-phenylbutyrate-treated IB3–1 cystic fibrosis bronchial epithelial cells. J Proteome Res 2006; 5: 562571.
  • 12
    Wright JM, Zeitlin PL, Cebotaru L, Guggino SE, Guggino WB. Gene expression profile analysis of 4-phenylbutyrate treatment of IB3–1 bronchial epithelial cell line demonstrates a major influence on heat-shock proteins. Physiol Genomics 2004; 16: 204211.
  • 13
    Kipp H, Arias IM. Intracellular trafficking and regulation of canalicular ATP-binding cassette transporters. Semin Liver Dis 2000; 20: 339351.
  • 14
    Keitel V, Kartenbeck J, Nies AT, Spring H, Brom M, Keppler D. Impaired protein maturation of the conjugate export pump multidrug resistance protein 2 as a consequence of a deletion mutation in Dubin-Johnson syndrome. HEPATOLOGY 2000; 32: 13171328.
  • 15
    Hashimoto K, Uchiumi T, Konno T, Ebihara T, Nakamura T, Wada M, et al. Trafficking and functional defects by mutations of the ATP-binding domains in MRP2 in patients with Dubin-Johnson syndrome. HEPATOLOGY 2002; 36: 12361245.