• 1
    Juliano RL, Ling V. A surface glycoprotein modulating drug permeability in Chinese hamster ovary cell mutants. Biochim Biophys Acta 1976; 455: 15262.
  • 2
    Ueda K, Cardarelli C, Gottesman MM, Pastan I. Expression of a full-length cDNA for the human ‘MDR1’ gene confers resistance to colchicine, doxorubicin, and vinblastine. Proc Natl Acad Sci USA 1987; 84: 30048.
  • 3
    Gottesman MM, Pastan I. Biochemistry of multidrug resistance mediated by the multidrug transporter. Annu Rev Biochem 1993; 62: 385427.
  • 4
    Sparreboom A, Van Asperen J, Mayer U et al . Limited oral bioavailability and active epithelial excretion of paclitaxel (Taxol) caused by P-glycoprotein in the intestine. Proc Natl Acad Sci USA 1997; 94: 20315.
  • 5
    Ambudkar SV, Dey S, Hrycyna CA, Ramachandra M, Pastan I, Gottesman MM. Biochemical, cellular, and pharmacological aspects of the multidrug transporter. Annu Rev Pharmacol Toxicol 1999; 39: 36198.
  • 6
    Greiner B, Eichelbaum M, Fritz P et al . The role of intestinal P–glycoprotein in the interaction of digoxin and rifampin. J Clin Invest 1999; 104: 14753.
  • 7
    Chen CJ, Chin JE, Ueda K et al . Internal duplication and homology with bacterial transport proteins in the mdr1 (P-glycoprotein) gene from multidrug-resistant human cells. Cell 1986; 47: 3819.
  • 8
    Urbatsch IL, Sankaran B, Bhagat S, Senior AE. Both P-glycoprotein nucleotide-binding sites are catalytically active. J Biol Chem 1995; 270: 26 95661.
  • 9
    Takada Y, Yamada K, Taguchi Y et al . Non-equivalent cooperation between the two nucleotide-binding folds of P-glycoprotein. Biochim Biophys Acta 1998; 14: 1316.
  • 10
    Ambudkar SV, Cardarelli CO, Pashinsky I, Stein WD. Relation between the turnover number for vinblastine transport and for vinblastine-stimulated ATP hydrolysis by human P-glycoprotein. J Biol Chem 1997; 272: 21 1606.
  • 11
    Eytan GD, Regev R, Assaraf YG. Functional reconstitution of P-glycoprotein reveals an apparent near stoichiometric drug transport to ATP hydrolysis. J Biol Chem 1996; 271: 31728.
  • 12
    Smit JJ, Schinkel AH, Oude Elferink RP et al . Homozygous disruption of the murine mdr2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease. Cell 1993; 75: 45162.
  • 13
    Inaba M, Sakurai Y. Enhanced efflux of actinomycin D, vincristine, and vinblastine in adriamycin-resistant subline of P388 leukemia. Cancer Lett 1979; 8: 11115.
  • 14
    Ueda K, Cornwell MM, Gottesman MM et al . The mdr1 gene, responsible for multidrug-resistance, codes for P-glycoprotein. Biochem Biophys Res Commun 1986; 141: 95662.
  • 15
    Gros P, Ben Neriah YB, Croop JM, Housman DE. Isolation and expression of a complementary DNA that confers multidrug resistance. Nature 1986; 323: 72831.
  • 16
    Pastan I, Gottesman MM, Ueda K, Lovelace E, Rutherford AV, Willingham MC. A retrovirus carrying an MDR1 cDNA confers multidrug resistance and polarized expression of P-glycoprotein in MDCK cells. Proc Natl Acad Sci USA 1988; 85: 448690.
  • 17
    Kioka N, Tsubota J, Kakehi Y et al . P-glycoprotein gene (MDR1) cDNA from human adrenal: normal P-glycoprotein carries Gly185 with an altered pattern of multidrug resistance. Biochem Biophys Res Commun 1989; 162: 22431.
  • 18
    Choi KH, Chen CJ, Kriegler M, Roninson IB. An altered pattern of cross-resistance in multidrug-resistant human cells results from spontaneous mutations in the mdr1 (P-glycoprotein) gene. Cell 1988; 53: 51929.
  • 19
    Gros P, Croop J, Housman D. Mammalian multidrug resistance gene: complete cDNA sequence indicates strong homology to bacterial transport protein. Cell 1986; 47: 37180.
  • 20
    Dean M, Rzhetsky A, Allikmets R. The human ATP-binding cassette (ABC) transporter superfamily. Genome Res 2001; 11: 115666.
  • 21
    Brooks-Wilson A, Marcil M, Clee S et al . Mutations in ABC1 in Tangier disease and familial high-density lipoprotein deficiency. Nat Genet 1999; 22: 33645.
  • 22
    Bodzioch M, Orso E, Klucken J et al . The gene encoding ATP-binding cassette transporter 1 is mutated in Tangier disease. Nat Genet 1999; 22: 34751.
  • 23
    Rust S, Rosier M, Funke H et al . Tangier disease is caused by mutations in the gene encoding ATP-binding cassette transporter 1. Nat Genet 1999; 22: 3525.
  • 24
    Shulenin S, Nogee LM, Annilo T, Wert SE, Whitsett JA, Dean M. ABCA3 gene mutations in newborns with fatal surfactant deficiency. N Engl J Med 2004; 350: 1296303.
  • 25
    Allikmets R, Singh N, Sun H et al . A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy. Nat Genet 1997; 15: 23646.
  • 26
    Berge KE, Tian H, Graf GA et al . Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters. Science 2000; 290: 17715.
  • 27
    Ueda K, Matsuo M, Tanabe K, Morita K, Kioka N, Amachi T. Comparative aspects of the function and mechanism of SUR1 and MDR1 proteins. Biochim Biophys Acta 1999; 1461: 30513.
  • 28
    Welsh MJ, Anderson MP, Rich DP et al . Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation. Neuron 1992; 8: 8219.
  • 29
    Riordan JR. The cystic fibrosis transmembrane conductance regulator. Annu Rev Physiol 1993; 55: 60930.
  • 30
    Gadsby DC, Nagel G, Hwang TC. The CFTR chloride channel of mammalian heart. Annu Rev Physiol 1995; 57: 387416.
  • 31
    Tsui LC, Buchwald M. Biochemical and molecular genetics of cystic fibrosis. Adv Hum Genet 1991; 20: 153266, 311–12.
  • 32
    Aguilar-Bryan L, Nichols CG, Wechsler SW et al . Cloning of the beta cell high-affinity sulfonylurea receptor: a regulator of insulin secretion. Science 1995; 268: 4236.
  • 33
    Clement JP 4th, Kunjilwar K, Gonzalez G et al . Association and stoichiometry of K (ATP) channel subunits. Neuron 1997; 18: 82738.
  • 34
    Inagaki N, Gonoi T, Seino S. Subunit stoichiometry of the pancreatic beta-cell ATP-sensitive K+ channel. FEBS Lett 1997; 409: 2326.
  • 35
    Mikhailov MV, Campbell JD De Wet H et al . 3-D structural and functional characterization of the purified KATP channel complex Kir6.2-SUR1. Embo J 2005; 24: 416675.
  • 36
    Matsuo M, Tanabe K, Kioka N, Amachi T, Ueda K. Different binding properties and affinities for ATP and ADP among sulfonylurea receptor subtypes, SUR1, SUR2A, and SUR2B. J Biol Chem 2000; 275: 2875763.
  • 37
    Ueda K, Komine J, Matsuo M, Seino S, Amachi T. Cooperative binding of ATP and MgADP in the sulfonylurea receptor is modulated by glibenclamide. Proc Natl Acad Sci USA 1999; 96: 126872.
  • 38
    Shitan N, Bazin I, Dan K et al . Involvement of CjMDR1, a plant multidrug-resistance-type ATP-binding cassette protein, in alkaloid transport in Coptis japonica. Proc Natl Acad Sci USA 2003; 100: 7516.
  • 39
    Cole SP, Bhardwaj G, Gerlach JH et al . Overexpression of a transporter gene in a multidrug-resistant human lung cancer cell line. Science 1992; 258: 16504.
  • 40
    Gerloff T, Stieger B, Hagenbuch B et al . The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver. J Biol Chem 1998; 273: 10 04650.
  • 41
    Tanaka AR, Abe-Dohmae S, Ohnishi T et al . Effects of mutations of ABCA1 in the first extracellular domain on subcellular trafficking and ATP binding/hydrolysis. J Biol Chem 2003; 278: 881519.
  • 42
    Mack JT, Beljanski V, Soulika AM et al . ‘Skittish’ Abca2 knockout mice display tremor, hyperactivity, and abnormal myelin ultrastructure in the central nervous system. Mol Cell Biol 2007; 27: 4453.
  • 43
    Nagata K, Yamamoto A, Ban N et al . Human ABCA3, a product of a responsible gene for abca3 for fatal surfactant deficiency in newborns, exhibits unique ATP hydrolysis activity and generates intracellular multilamellar vesicles. Biochem Biophys Res Commun 2004; 324: 2628.
  • 44
    Oude Elferink RP, Ottenhoff R, Van Wijland M, Frijters CM, Van Nieuwkerk C, Groen AK. Uncoupling of biliary phospholipid and cholesterol secretion in mice with reduced expression of mdr2 P-glycoprotein. J Lipid Res 1996; 37: 106575.
  • 45
    Smith AJ, De Vree JM, Ottenhoff R, Oude Elferink RP, Schinkel AH, Borst P. Hepatocyte-specific expression of the human MDR3 P-glycoprotein gene restores the biliary phosphatidylcholine excretion absent in Mdr2 (–/–) mice. Hepatology 1998; 28: 5306.
  • 46
    Lammert F, Wang DQ, Hillebrandt S et al . Spontaneous cholecysto- and hepatolithiasis in Mdr2–/– mice: a model for low phospholipid-associated cholelithiasis. Hepatology 2004; 39: 11728.
  • 47
    Fickert P, Zollner G, Fuchsbichler A et al . Ursodeoxycholic acid aggravates bile infarcts in bile duct-ligated and Mdr2 knockout mice via disruption of cholangioles. Gastroenterology 2002; 123: 123851.
  • 48
    Jacquemin E, De Vree JM, Cresteil D et al . The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood. Gastroenterology 2001; 120: 144858.
  • 49
    Oude Elferink RP, Paulusma CC. Function and pathophysiological importance of ABCB4 (MDR3 P-glycoprotein). Pflugers Arch 2007; 453: 60110.
  • 50
    Ruetz S, Gros P. Phosphatidylcholine translocase. a physiological role for the mdr2 gene. Cell 1994; 77: 107181.
  • 51
    Van Helvoort A, Smith AJ, Sprong H et al . MDR1 P-glycoprotein is a lipid translocase of broad specificity, while MDR3 P-glycoprotein specifically translocates phosphatidylcholine. Cell 1996; 87: 50717.
  • 52
    Smith AJ, Timmermans-Hereijgers JL, Roelofsen B et al . The human MDR3 P-glycoprotein promotes translocation of phosphatidylcholine through the plasma membrane of fibroblasts from transgenic mice. FEBS Lett 1994; 354: 2636.
  • 53
    Morita S-Y, Kobayashi A, Takanezawa Y et al . Bile salt-dependent efflux of cellular phospholipids mediated by ATP binding cassette protein B4. Hepatology 2007; May 23; [Epub ahead of print].
  • 54
    Matsuoka K, Maeda M, Moroi Y. Micelle formation of sodium glyco- and taurocholates and sodium glyco- and taurodeoxycholates and solubilization of cholesterol into their micelles. Colloids Surfaces, B Biointerfaces 2003; 32: 8795.
  • 55
    Heuman DM. Quantitative estimation of the hydrophilic-hydrophobic balance of mixed bile salt solutions. J Lipid Res 1989; 30: 71930.
  • 56
    Van Erpecum KJ. Biliary lipids, water and cholesterol gallstones. Biol Cell 2005; 97: 81522.
  • 57
    Van Der Bliek AM, Kooiman PM, Schneider C, Borst P. Sequence of mdr3 cDNA encoding a human P-glycoprotein. Gene 1988; 71: 40111.
  • 58
    Kino K, Taguchi Y, Yamada K, Komano T, Ueda K. Aureobasidin A, an antifungal cyclic depsipeptide antibiotic, is a substrate for both human MDR1 and MDR2/P-glycoproteins. FEBS Lett 1996; 399: 2932.
  • 59
    Smith AJ, Van Helvoort A, Van Meer G et al . MDR3 P-glycoprotein, a phosphatidylcholine translocase, transports several cytotoxic drugs and directly interacts with drugs as judged by interference with nucleotide trapping. J Biol Chem 2000; 275: 23 5309.
  • 60
    Rothnie A, Theron D, Soceneantu L et al . The importance of cholesterol in maintenance of P-glycoprotein activity and its membrane perturbing influence. Eur Biophys J 2001; 30: 43042.
  • 61
    Gayet L, Dayan G, Barakat S et al . Control of P-glycoprotein activity by membrane cholesterol amounts and their relation to multidrug resistance in human CEM leukemia cells. Biochemistry 2005; 44: 4499509.
  • 62
    Garrigues A, Escargueil AE, Orlowski S. The multidrug transporter, P-glycoprotein, actively mediates cholesterol redistribution in the cell membrane. Proc Natl Acad Sci USA 2002; 99: 1034752.
  • 63
    Troost J, Albermann N, Emil Haefeli W, Weiss J. Cholesterol modulates P-glycoprotein activity in human peripheral blood mononuclear cells. Biochem Biophys Res Commun 2004; 316: 70511.
  • 64
    Troost J, Lindenmaier H, Haefeli WE, Weiss J. Modulation of cellular cholesterol alters P-glycoprotein activity in multidrug-resistant cells. Mol Pharmacol 2004; 66: 13329.
  • 65
    Kimura Y, Kioka N, Kato H, Matsuo M, Ueda K. Modulation of drug-stimulated ATPase activity of human MDR1/P-glycoprotein by cholesterol. Biochem J 2007; 401: 597605.
  • 66
    Martin C, Berridge G, Higgins CF, Mistry P, Charlton P, Callaghan R. Communication between multiple drug binding sites on P-glycoprotein. Mol Pharmacol 2000; 58: 62432.
  • 67
    Shapiro AB, Fox K, Lam P, Ling V. Stimulation of P-glycoprotein-mediated drug transport by prazosin and progesterone. Evidence for a third drug-binding site. Eur J Biochem 1999; 259: 84150.
  • 68
    Loo TW, Bartlett MC, Clarke DM. Simultaneous binding of two different drugs in the binding pocket of the human multidrug resistance P-glycoprotein. J Biol Chem 2003; 278: 3970610.
  • 69
    Loo TW, Clarke DM. Location of the rhodamine-binding site in the human multidrug resistance P-glycoprotein. J Biol Chem 2002; 277: 44 3328.
  • 70
    Taguchi Y, Kino K, Morishima M, Komano T, Kane SE, Ueda K. Alteration of substrate specificity by mutations at the His61 position in predicted transmembrane domain 1 of human MDR1/P-glycoprotein. Biochemistry 1997; 36: 88839.
  • 71
    Taguchi Y, Morishima M, Komano T, Ueda K. Amino acid substitutions in the first transmembrane domain (TM1) of P-glycoprotein that alter substrate specificity. FEBS Lett 1997; 413: 1426.
  • 72
    Loo TW, Bartlett MC, Clarke DM. Transmembrane segment 1 of human P-glycoprotein contributes to the drug binding pocket. Biochem J 2006; 396: 53745.
  • 73
    Takahashi K, Kimura Y, Kioka N, Matsuo M, Ueda K. Purification and ATPase activity of human ABCA1. J Biol Chem 2006; 281: 10 7608.
  • 74
    Nagao K, Takahashi K, Hanada K, Kioka N, Matsuo M, Ueda K. Enhanced apoA-1-dependent cholesterol efflux by ABCA1 from sphingomyelin-deficient Chinese hamster ovary cells. J Biol Chem 2007; 282: 14 86874.
  • 75
    Kobayashi A, Takanezawa Y, Hirata T et al . Efflux of sphingomyelin, cholesterol, and phosphatidylcholine by ABCG1. J Lipid Res 2006; 47: 1791802.