메뉴 건너뛰기




Volumn 6, Issue 9, 2011, Pages

Expression and In Vivo rescue of human ABCC6 disease-causing mutants in mouse liver

Author keywords

[No Author keywords available]

Indexed keywords

4 PHENYLBUTYRIC ACID; ABC TRANSPORTER; ABCC6 PROTEIN; UNCLASSIFIED DRUG; ABCC6 PROTEIN, HUMAN; MULTIDRUG RESISTANCE PROTEIN;

EID: 80052827583     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0024738     Document Type: Article
Times cited : (61)

References (53)
  • 1
    • 43149100350 scopus 로고    scopus 로고
    • An alternative splice variant in Abcc6, the gene causing dystrophic calcification, leads to protein deficiency in C3H/He mice
    • Aherrahrou Z, Doehring LC, Ehlers EM, Liptau H, Depping R, et al. (2008) An alternative splice variant in Abcc6, the gene causing dystrophic calcification, leads to protein deficiency in C3H/He mice. J Biol Chem 283: 7608-7615.
    • (2008) J Biol Chem , vol.283 , pp. 7608-7615
    • Aherrahrou, Z.1    Doehring, L.C.2    Ehlers, E.M.3    Liptau, H.4    Depping, R.5
  • 3
    • 18844465976 scopus 로고    scopus 로고
    • Mutations in a gene encoding an ABC transporter cause pseudoxanthoma elasticum
    • Le Saux O, Urban Z, Tschuch C, Csiszar K, Bacchelli B, et al. (2000) Mutations in a gene encoding an ABC transporter cause pseudoxanthoma elasticum. Nat Genet 25: 223-227.
    • (2000) Nat Genet , vol.25 , pp. 223-227
    • Le Saux, O.1    Urban, Z.2    Tschuch, C.3    Csiszar, K.4    Bacchelli, B.5
  • 4
    • 34248390171 scopus 로고    scopus 로고
    • Identification of Abcc6 as the major causal gene for dystrophic cardiac calcification in mice through integrative genomics
    • Meng H, Vera I, Che N, Wang X, Wang SS, et al. (2007) Identification of Abcc6 as the major causal gene for dystrophic cardiac calcification in mice through integrative genomics. Proc Natl Acad Sci U S A 104: 4530-4535.
    • (2007) Proc Natl Acad Sci U S A , vol.104 , pp. 4530-4535
    • Meng, H.1    Vera, I.2    Che, N.3    Wang, X.4    Wang, S.S.5
  • 5
    • 0034705145 scopus 로고    scopus 로고
    • Pseudoxanthoma elasticum: mutations in the MRP6 gene encoding a transmembrane ATP-binding cassette (ABC) transporter
    • Ringpfeil F, Lebwohl MG, Christiano AM, Uitto J, (2000) Pseudoxanthoma elasticum: mutations in the MRP6 gene encoding a transmembrane ATP-binding cassette (ABC) transporter. Proc Natl Acad Sci U S A 97: 6001-6006.
    • (2000) Proc Natl Acad Sci U S A , vol.97 , pp. 6001-6006
    • Ringpfeil, F.1    Lebwohl, M.G.2    Christiano, A.M.3    Uitto, J.4
  • 7
    • 28844498292 scopus 로고    scopus 로고
    • Pseudoxanthoma elasticum: a clinical, pathophysiological and genetic update including 11 novel ABCC6 mutations
    • Chassaing N, Martin L, Calvas P, Le Bert M, Hovnanian A, (2005) Pseudoxanthoma elasticum: a clinical, pathophysiological and genetic update including 11 novel ABCC6 mutations. J Med Genet 42: 881-892.
    • (2005) J Med Genet , vol.42 , pp. 881-892
    • Chassaing, N.1    Martin, L.2    Calvas, P.3    Le Bert, M.4    Hovnanian, A.5
  • 8
    • 57649232758 scopus 로고    scopus 로고
    • Pseudoxanthoma elasticum: clinical phenotypes, molecular genetics and putative pathomechanisms
    • Li Q, Jiang Q, Pfendner E, Varadi A, Uitto J, (2009) Pseudoxanthoma elasticum: clinical phenotypes, molecular genetics and putative pathomechanisms. Exp Dermatol 18: 1-11.
    • (2009) Exp Dermatol , vol.18 , pp. 1-11
    • Li, Q.1    Jiang, Q.2    Pfendner, E.3    Varadi, A.4    Uitto, J.5
  • 9
    • 76949097206 scopus 로고    scopus 로고
    • The R1141X loss-of-function mutation of the ABCC6 gene is a strong genetic risk factor for coronary artery disease
    • Koblos G, Andrikovics H, Prohaszka Z, Tordai A, Varadi A, et al. (2010) The R1141X loss-of-function mutation of the ABCC6 gene is a strong genetic risk factor for coronary artery disease. Genet Test Mol Biomarkers 14: 75-78.
    • (2010) Genet Test Mol Biomarkers , vol.14 , pp. 75-78
    • Koblos, G.1    Andrikovics, H.2    Prohaszka, Z.3    Tordai, A.4    Varadi, A.5
  • 10
    • 41149104552 scopus 로고    scopus 로고
    • Heterozygosity for a single mutation in the ABCC6 gene may closely mimic PXE: consequences of this phenotype overlap for the definition of PXE
    • Martin L, Maitre F, Bonicel P, Daudon P, Verny C, et al. (2008) Heterozygosity for a single mutation in the ABCC6 gene may closely mimic PXE: consequences of this phenotype overlap for the definition of PXE. Arch Dermatol 144: 301-306.
    • (2008) Arch Dermatol , vol.144 , pp. 301-306
    • Martin, L.1    Maitre, F.2    Bonicel, P.3    Daudon, P.4    Verny, C.5
  • 11
    • 0037072447 scopus 로고    scopus 로고
    • Frequent mutation in the ABCC6 gene (R1141X) is associated with a strong increase in the prevalence of coronary artery disease
    • Trip MD, Smulders YM, Wegman JJ, Hu X, Boer JM, et al. (2002) Frequent mutation in the ABCC6 gene (R1141X) is associated with a strong increase in the prevalence of coronary artery disease. Circulation 106: 773-775.
    • (2002) Circulation , vol.106 , pp. 773-775
    • Trip, M.D.1    Smulders, Y.M.2    Wegman, J.J.3    Hu, X.4    Boer, J.M.5
  • 12
    • 77954896821 scopus 로고    scopus 로고
    • The ERK1/2-hepatocyte nuclear factor 4alpha axis regulates human ABCC6 gene expression in hepatocytes
    • de Boussac H, Ratajewski M, Sachrajda I, Koblos G, Tordai A, et al. (2010) The ERK1/2-hepatocyte nuclear factor 4alpha axis regulates human ABCC6 gene expression in hepatocytes. J Biol Chem 285: 22800-22808.
    • (2010) J Biol Chem , vol.285 , pp. 22800-22808
    • de Boussac, H.1    Ratajewski, M.2    Sachrajda, I.3    Koblos, G.4    Tordai, A.5
  • 13
    • 21444432422 scopus 로고    scopus 로고
    • Identification of a DNA methylation-dependent activator sequence in the pseudoxanthoma elasticum gene, ABCC6
    • Aranyi T, Ratajewski M, Bardoczy V, Pulaski L, Bors A, et al. (2005) Identification of a DNA methylation-dependent activator sequence in the pseudoxanthoma elasticum gene, ABCC6. J Biol Chem 280: 18643-18650.
    • (2005) J Biol Chem , vol.280 , pp. 18643-18650
    • Aranyi, T.1    Ratajewski, M.2    Bardoczy, V.3    Pulaski, L.4    Bors, A.5
  • 14
    • 0037053355 scopus 로고    scopus 로고
    • Loss of ATP-dependent transport activity in pseudoxanthoma elasticum-associated mutants of human ABCC6 (MRP6)
    • Ilias A, Urban Z, Seidl TL, Le Saux O, Sinko E, et al. (2002) Loss of ATP-dependent transport activity in pseudoxanthoma elasticum-associated mutants of human ABCC6 (MRP6). J Biol Chem 277: 16860-16867.
    • (2002) J Biol Chem , vol.277 , pp. 16860-16867
    • Ilias, A.1    Urban, Z.2    Seidl, T.L.3    Le Saux, O.4    Sinko, E.5
  • 15
    • 0036829109 scopus 로고    scopus 로고
    • Characterization of the drug resistance and transport properties of multidrug resistance protein 6 (MRP6, ABCC6)
    • Belinsky MG, Chen ZS, Shchaveleva I, Zeng H, Kruh GD, (2002) Characterization of the drug resistance and transport properties of multidrug resistance protein 6 (MRP6, ABCC6). Cancer Res 62: 6172-6177.
    • (2002) Cancer Res , vol.62 , pp. 6172-6177
    • Belinsky, M.G.1    Chen, Z.S.2    Shchaveleva, I.3    Zeng, H.4    Kruh, G.D.5
  • 16
    • 0038648741 scopus 로고    scopus 로고
    • The distribution of Abcc6 in normal mouse tissues suggests multiple functions for this ABC transporter
    • Beck K, Hayashi K, Nishiguchi B, Le Saux O, Hayashi M, et al. (2003) The distribution of Abcc6 in normal mouse tissues suggests multiple functions for this ABC transporter. J Histochem Cytochem 51: 887-902.
    • (2003) J Histochem Cytochem , vol.51 , pp. 887-902
    • Beck, K.1    Hayashi, K.2    Nishiguchi, B.3    Le Saux, O.4    Hayashi, M.5
  • 18
    • 33745213755 scopus 로고    scopus 로고
    • Serum factors from pseudoxanthoma elasticum patients alter elastic fiber formation in vitro
    • Le Saux O, Bunda S, VanWart CM, Douet V, Got L, et al. (2006) Serum factors from pseudoxanthoma elasticum patients alter elastic fiber formation in vitro. J Invest Dermatol 126: 1497-1505.
    • (2006) J Invest Dermatol , vol.126 , pp. 1497-1505
    • Le Saux, O.1    Bunda, S.2    VanWart, C.M.3    Douet, V.4    Got, L.5
  • 20
    • 76649133470 scopus 로고    scopus 로고
    • Parabiotic heterogenetic pairing of Abcc6-/-/Rag1-/- mice and their wild-type counterparts halts ectopic mineralization in a murine model of pseudoxanthoma elasticum
    • Jiang Q, Oldenburg R, Otsuru S, Grand-Pierre AE, Horwitz EM, et al. (2010) Parabiotic heterogenetic pairing of Abcc6-/-/Rag1-/- mice and their wild-type counterparts halts ectopic mineralization in a murine model of pseudoxanthoma elasticum. Am J Pathol 176: 1855-1862.
    • (2010) Am J Pathol , vol.176 , pp. 1855-1862
    • Jiang, Q.1    Oldenburg, R.2    Otsuru, S.3    Grand-Pierre, A.E.4    Horwitz, E.M.5
  • 21
    • 0034835028 scopus 로고    scopus 로고
    • A spectrum of ABCC6 mutations is responsible for pseudoxanthoma elasticum
    • Le Saux O, Beck K, Sachsinger C, Silvestri C, Treiber C, et al. (2001) A spectrum of ABCC6 mutations is responsible for pseudoxanthoma elasticum. Am J Hum Genet 69: 749-764.
    • (2001) Am J Hum Genet , vol.69 , pp. 749-764
    • Le Saux, O.1    Beck, K.2    Sachsinger, C.3    Silvestri, C.4    Treiber, C.5
  • 22
    • 35348906644 scopus 로고    scopus 로고
    • Mutation detection in the ABCC6 gene and genotype-phenotype analysis in a large international case series affected by pseudoxanthoma elasticum
    • Pfendner EG, Vanakker OM, Terry SF, Vourthis S, McAndrew PE, et al. (2007) Mutation detection in the ABCC6 gene and genotype-phenotype analysis in a large international case series affected by pseudoxanthoma elasticum. J Med Genet 44: 621-628.
    • (2007) J Med Genet , vol.44 , pp. 621-628
    • Pfendner, E.G.1    Vanakker, O.M.2    Terry, S.F.3    Vourthis, S.4    McAndrew, P.E.5
  • 23
  • 24
    • 0030809817 scopus 로고    scopus 로고
    • In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR
    • Rubenstein RC, Egan ME, Zeitlin PL, (1997) 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 100: 2457-2465.
    • (1997) J Clin Invest , vol.100 , pp. 2457-2465
    • Rubenstein, R.C.1    Egan, M.E.2    Zeitlin, P.L.3
  • 25
    • 0031889082 scopus 로고    scopus 로고
    • A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function
    • Rubenstein RC, Zeitlin PL, (1998) 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 157: 484-490.
    • (1998) Am J Respir Crit Care Med , vol.157 , pp. 484-490
    • Rubenstein, R.C.1    Zeitlin, P.L.2
  • 26
    • 0034099743 scopus 로고    scopus 로고
    • Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR
    • Rubenstein RC, Zeitlin PL, (2000) Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR. Am J Physiol Cell Physiol 278: C259-267.
    • (2000) Am J Physiol Cell Physiol , vol.278
    • Rubenstein, R.C.1    Zeitlin, P.L.2
  • 27
    • 33646897427 scopus 로고    scopus 로고
    • Functional and trafficking defects in ATP binding cassette A3 mutants associated with respiratory distress syndrome
    • Cheong N, Madesh M, Gonzales LW, Zhao M, Yu K, et al. (2006) Functional and trafficking defects in ATP binding cassette A3 mutants associated with respiratory distress syndrome. J Biol Chem 281: 9791-9800.
    • (2006) J Biol Chem , vol.281 , pp. 9791-9800
    • Cheong, N.1    Madesh, M.2    Gonzales, L.W.3    Zhao, M.4    Yu, K.5
  • 28
    • 34247172594 scopus 로고    scopus 로고
    • 4-Phenylbutyrate restores the functionality of a misfolded mutant low-density lipoprotein receptor
    • Tveten K, Holla OL, Ranheim T, Berge KE, Leren TP, et al. (2007) 4-Phenylbutyrate restores the functionality of a misfolded mutant low-density lipoprotein receptor. FEBS J 274: 1881-1893.
    • (2007) FEBS J , vol.274 , pp. 1881-1893
    • Tveten, K.1    Holla, O.L.2    Ranheim, T.3    Berge, K.E.4    Leren, T.P.5
  • 29
    • 34250339695 scopus 로고    scopus 로고
    • 4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps
    • Hayashi H, Sugiyama Y, (2007) 4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps. Hepatology 45: 1506-1516.
    • (2007) Hepatology , vol.45 , pp. 1506-1516
    • Hayashi, H.1    Sugiyama, Y.2
  • 30
    • 36048976850 scopus 로고    scopus 로고
    • Levels of plasma membrane expression in progressive and benign mutations of the bile salt export pump (Bsep/Abcb11) correlate with severity of cholestatic diseases
    • Lam P, Pearson CL, Soroka CJ, Xu S, Mennone A, et al. (2007) Levels of plasma membrane expression in progressive and benign mutations of the bile salt export pump (Bsep/Abcb11) correlate with severity of cholestatic diseases. Am J Physiol Cell Physiol 293: C1709-1716.
    • (2007) Am J Physiol Cell Physiol , vol.293
    • Lam, P.1    Pearson, C.L.2    Soroka, C.J.3    Xu, S.4    Mennone, A.5
  • 31
    • 58849138006 scopus 로고    scopus 로고
    • Short-chain ubiquitination is associated with the degradation rate of a cell-surface-resident bile salt export pump (BSEP/ABCB11)
    • Hayashi H, Sugiyama Y, (2009) Short-chain ubiquitination is associated with the degradation rate of a cell-surface-resident bile salt export pump (BSEP/ABCB11). Mol Pharmacol 75: 143-150.
    • (2009) Mol Pharmacol , vol.75 , pp. 143-150
    • Hayashi, H.1    Sugiyama, Y.2
  • 32
    • 73149122801 scopus 로고    scopus 로고
    • Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin
    • van den Berghe PV, Stapelbroek JM, Krieger E, de Bie P, van de Graaf SF, et al. (2009) Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin. Hepatology 50: 1783-1795.
    • (2009) Hepatology , vol.50 , pp. 1783-1795
    • van den Berghe, P.V.1    Stapelbroek, J.M.2    Krieger, E.3    de Bie, P.4    van de Graaf, S.F.5
  • 33
    • 73449147315 scopus 로고    scopus 로고
    • Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate
    • van der Velden LM, Stapelbroek JM, Krieger E, van den Berghe PV, Berger R, et al. (2010) Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate. Hepatology 51: 286-296.
    • (2010) Hepatology , vol.51 , pp. 286-296
    • van der Velden, L.M.1    Stapelbroek, J.M.2    Krieger, E.3    van den Berghe, P.V.4    Berger, R.5
  • 34
    • 79953221452 scopus 로고    scopus 로고
    • In vitro recovery of ATP-sensitive potassium channels in beta-cells from patients with congenital hyperinsulinism of infancy
    • Powell PD, Bellanne-Chantelot C, Flanagan SE, Ellard S, Rooman R, et al. (2011) In vitro recovery of ATP-sensitive potassium channels in beta-cells from patients with congenital hyperinsulinism of infancy. Diabetes 60: 1223-1228.
    • (2011) Diabetes , vol.60 , pp. 1223-1228
    • Powell, P.D.1    Bellanne-Chantelot, C.2    Flanagan, S.E.3    Ellard, S.4    Rooman, R.5
  • 35
    • 0026710710 scopus 로고
    • Increased fetal hemoglobin in patients receiving sodium 4-phenylbutyrate
    • Dover GJ, Brusilow S, Samid D, (1992) Increased fetal hemoglobin in patients receiving sodium 4-phenylbutyrate. N Engl J Med 327: 569-570.
    • (1992) N Engl J Med , vol.327 , pp. 569-570
    • Dover, G.J.1    Brusilow, S.2    Samid, D.3
  • 36
    • 0029786498 scopus 로고    scopus 로고
    • Long-term treatment of girls with ornithine transcarbamylase deficiency
    • Maestri NE, Brusilow SW, Clissold DB, Bassett SS, (1996) Long-term treatment of girls with ornithine transcarbamylase deficiency. N Engl J Med 335: 855-859.
    • (1996) N Engl J Med , vol.335 , pp. 855-859
    • Maestri, N.E.1    Brusilow, S.W.2    Clissold, D.B.3    Bassett, S.S.4
  • 37
    • 0027078611 scopus 로고
    • A short-term trial of butyrate to stimulate fetal-globin-gene expression in the beta-globin disorders
    • Perrine SP, Ginder GD, Faller DV, Dover GH, Ikuta T, et al. (1993) A short-term trial of butyrate to stimulate fetal-globin-gene expression in the beta-globin disorders. N Engl J Med 328: 81-86.
    • (1993) N Engl J Med , vol.328 , pp. 81-86
    • Perrine, S.P.1    Ginder, G.D.2    Faller, D.V.3    Dover, G.H.4    Ikuta, T.5
  • 38
    • 34248376062 scopus 로고    scopus 로고
    • Sodium 4-phenylbutyrate acts as a chemical chaperone on misfolded myocilin to rescue cells from endoplasmic reticulum stress and apoptosis
    • Yam GH, Gaplovska-Kysela K, Zuber C, Roth J, (2007) Sodium 4-phenylbutyrate acts as a chemical chaperone on misfolded myocilin to rescue cells from endoplasmic reticulum stress and apoptosis. Invest Ophthalmol Vis Sci 48: 1683-1690.
    • (2007) Invest Ophthalmol Vis Sci , vol.48 , pp. 1683-1690
    • Yam, G.H.1    Gaplovska-Kysela, K.2    Zuber, C.3    Roth, J.4
  • 39
    • 0035874464 scopus 로고    scopus 로고
    • Role of glycine-534 and glycine-1179 of human multidrug resistance protein (MDR1) in drug-mediated control of ATP hydrolysis
    • Szakacs G, Ozvegy C, Bakos E, Sarkadi B, Varadi A, (2001) Role of glycine-534 and glycine-1179 of human multidrug resistance protein (MDR1) in drug-mediated control of ATP hydrolysis. Biochem J 356: 71-75.
    • (2001) Biochem J , vol.356 , pp. 71-75
    • Szakacs, G.1    Ozvegy, C.2    Bakos, E.3    Sarkadi, B.4    Varadi, A.5
  • 40
    • 0026722467 scopus 로고
    • Expression of the human multidrug resistance cDNA in insect cells generates a high activity drug-stimulated membrane ATPase
    • Sarkadi B, Price EM, Boucher RC, Germann UA, Scarborough GA, (1992) Expression of the human multidrug resistance cDNA in insect cells generates a high activity drug-stimulated membrane ATPase. J Biol Chem 267: 4854-4858.
    • (1992) J Biol Chem , vol.267 , pp. 4854-4858
    • Sarkadi, B.1    Price, E.M.2    Boucher, R.C.3    Germann, U.A.4    Scarborough, G.A.5
  • 41
    • 0033610801 scopus 로고    scopus 로고
    • Functional multidrug resistance protein (MRP1) lacking the N-terminal transmembrane domain
    • Bakos E, Evers R, Szakacs G, Tusnady GE, Welker E, et al. (1998) Functional multidrug resistance protein (MRP1) lacking the N-terminal transmembrane domain. J Biol Chem 273: 32167-32175.
    • (1998) J Biol Chem , vol.273 , pp. 32167-32175
    • Bakos, E.1    Evers, R.2    Szakacs, G.3    Tusnady, G.E.4    Welker, E.5
  • 42
    • 0042093650 scopus 로고    scopus 로고
    • Subcellular localization and N-glycosylation of human ABCC6, expressed in MDCKII cells
    • Sinko E, Ilias A, Ujhelly O, Homolya L, Scheffer GL, et al. (2003) Subcellular localization and N-glycosylation of human ABCC6, expressed in MDCKII cells. Biochem Biophys Res Commun 308: 263-269.
    • (2003) Biochem Biophys Res Commun , vol.308 , pp. 263-269
    • Sinko, E.1    Ilias, A.2    Ujhelly, O.3    Homolya, L.4    Scheffer, G.L.5
  • 43
    • 0032805251 scopus 로고    scopus 로고
    • Hydrodynamics-based transfection in animals by systemic administration of plasmid DNA
    • Liu F, Song Y, Liu D, (1999) Hydrodynamics-based transfection in animals by systemic administration of plasmid DNA. Gene Ther 6: 1258-1266.
    • (1999) Gene Ther , vol.6 , pp. 1258-1266
    • Liu, F.1    Song, Y.2    Liu, D.3
  • 44
    • 1942537809 scopus 로고    scopus 로고
    • Hydroporation as the mechanism of hydrodynamic delivery
    • Zhang G, Gao X, Song YK, Vollmer R, Stolz DB, et al. (2004) Hydroporation as the mechanism of hydrodynamic delivery. Gene Ther 11: 675-682.
    • (2004) Gene Ther , vol.11 , pp. 675-682
    • Zhang, G.1    Gao, X.2    Song, Y.K.3    Vollmer, R.4    Stolz, D.B.5
  • 45
    • 0034007737 scopus 로고    scopus 로고
    • Transport function and hepatocellular localization of mrp6 in rat liver
    • Madon J, Hagenbuch B, Landmann L, Meier PJ, Stieger B, (2000) Transport function and hepatocellular localization of mrp6 in rat liver. Mol Pharmacol 57: 634-641.
    • (2000) Mol Pharmacol , vol.57 , pp. 634-641
    • Madon, J.1    Hagenbuch, B.2    Landmann, L.3    Meier, P.J.4    Stieger, B.5
  • 46
    • 76649133303 scopus 로고    scopus 로고
    • Pseudoxanthoma elasticum: molecular genetics and putative pathomechanisms
    • Uitto J, Li Q, Jiang Q, (2010) Pseudoxanthoma elasticum: molecular genetics and putative pathomechanisms. J Invest Dermatol 130: 661-670.
    • (2010) J Invest Dermatol , vol.130 , pp. 661-670
    • Uitto, J.1    Li, Q.2    Jiang, Q.3
  • 47
    • 33645026944 scopus 로고    scopus 로고
    • Pseudoxanthoma elasticum: the end of the autosomal dominant segregation myth
    • Bergen AA, (2006) Pseudoxanthoma elasticum: the end of the autosomal dominant segregation myth. J Invest Dermatol 126: 704-705.
    • (2006) J Invest Dermatol , vol.126 , pp. 704-705
    • Bergen, A.A.1
  • 48
    • 0033166561 scopus 로고    scopus 로고
    • High levels of foreign gene expression in hepatocytes after tail vein injections of naked plasmid DNA
    • Zhang G, Budker V, Wolff JA, (1999) High levels of foreign gene expression in hepatocytes after tail vein injections of naked plasmid DNA. Hum Gene Ther 10: 1735-1737.
    • (1999) Hum Gene Ther , vol.10 , pp. 1735-1737
    • Zhang, G.1    Budker, V.2    Wolff, J.A.3
  • 49
    • 0037163124 scopus 로고    scopus 로고
    • Role of the N-terminal transmembrane region of the multidrug resistance protein MRP2 in routing to the apical membrane in MDCKII cells
    • Fernandez SB, Hollo Z, Kern A, Bakos E, Fischer PA, et al. (2002) Role of the N-terminal transmembrane region of the multidrug resistance protein MRP2 in routing to the apical membrane in MDCKII cells. J Biol Chem 277: 31048-31055.
    • (2002) J Biol Chem , vol.277 , pp. 31048-31055
    • Fernandez, S.B.1    Hollo, Z.2    Kern, A.3    Bakos, E.4    Fischer, P.A.5
  • 50
    • 0036303981 scopus 로고    scopus 로고
    • Hassles with taking out the garbage: aggravating aggresomes
    • Garcia-Mata R, Gao YS, Sztul E, (2002) Hassles with taking out the garbage: aggravating aggresomes. Traffic 3: 388-396.
    • (2002) Traffic , vol.3 , pp. 388-396
    • Garcia-Mata, R.1    Gao, Y.S.2    Sztul, E.3
  • 51
  • 52
    • 28344451849 scopus 로고    scopus 로고
    • Expression of a wild-type CFTR maintains the integrity of the biosynthetic/secretory pathway in human cystic fibrosis pancreatic duct cells
    • Hollande E, Salvador-Cartier C, Alvarez L, Fanjul M, (2005) Expression of a wild-type CFTR maintains the integrity of the biosynthetic/secretory pathway in human cystic fibrosis pancreatic duct cells. J Histochem Cytochem 53: 1539-1552.
    • (2005) J Histochem Cytochem , vol.53 , pp. 1539-1552
    • Hollande, E.1    Salvador-Cartier, C.2    Alvarez, L.3    Fanjul, M.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.