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Volumn 47, Issue C, 1999, Pages 351-380

Chapter 20 Epithelial Sodium Channels in Cystic Fibrosis

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EID: 0004704752     PISSN: 00702161     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S0070-2161(08)60968-5     Document Type: Article
Times cited : (5)

References (140)
  • 1
    • 0003064682 scopus 로고
    • Reduction of dimensionality in biological diffusion processes.
    • Rich A., and Davidson N. (Eds), Freeman, San Francisco
    • Adam G., and Delbriick M. Reduction of dimensionality in biological diffusion processes. In: Rich A., and Davidson N. (Eds). "Structural Chemistry and Molecular Biology. (1967), Freeman, San Francisco 198-215
    • (1967) "Structural Chemistry and Molecular Biology. , pp. 198-215
    • Adam, G.1    Delbriick, M.2
  • 2
    • 0030854398 scopus 로고    scopus 로고
    • Interactions between subunits of the human epithelial sodium channel
    • Adams C.M., Snyder P.M., and Welsh M.J. Interactions between subunits of the human epithelial sodium channel. J. Biol. Chem. 272 (1997) 27295-27300
    • (1997) J. Biol. Chem. , vol.272 , pp. 27295-27300
    • Adams, C.M.1    Snyder, P.M.2    Welsh, M.J.3
  • 3
    • 0020144768 scopus 로고
    • Diffusion of molecules on biological membranes of nonplanar form. A theoretical study
    • Aizenbud B.M., and Oershon N.D. Diffusion of molecules on biological membranes of nonplanar form. A theoretical study. Biophys. J. 38 (1982) 287-293
    • (1982) Biophys. J. , vol.38 , pp. 287-293
    • Aizenbud, B.M.1    Oershon, N.D.2
  • 4
    • 0022137173 scopus 로고
    • Diffusion of molecules on biological membranes of nonplanar form. II. Diffusion anisotropy
    • Aizenbud B.M., and Gershon N.D. Diffusion of molecules on biological membranes of nonplanar form. II. Diffusion anisotropy. Biophys. J. 48 (1985) 543-546
    • (1985) Biophys. J. , vol.48 , pp. 543-546
    • Aizenbud, B.M.1    Gershon, N.D.2
  • 6
    • 0023275376 scopus 로고
    • Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis
    • Alton E.W., Hay J.G., Munro C., and Geddes D.M. Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis. Thorax 42 (1987) 815-817
    • (1987) Thorax , vol.42 , pp. 815-817
    • Alton, E.W.1    Hay, J.G.2    Munro, C.3    Geddes, D.M.4
  • 9
    • 0025274624 scopus 로고
    • Acute and long-term amiloride inhalation in cystic fibrosis lung disease. A rational approach to cystic fibrosis therapy
    • App E.M., King M., Helfesrieder R., Kohler D., and Matthys H. Acute and long-term amiloride inhalation in cystic fibrosis lung disease. A rational approach to cystic fibrosis therapy. Am. Rev. Respire. Dis. 141 (1990) 605-612
    • (1990) Am. Rev. Respire. Dis. , vol.141 , pp. 605-612
    • App, E.M.1    King, M.2    Helfesrieder, R.3    Kohler, D.4    Matthys, H.5
  • 13
    • 0030775656 scopus 로고    scopus 로고
    • Decreased sodiumion absorption across nasal epithelium of very premature infants with respiratory distress syndrome
    • Barker P.M., Gowen C.W., Lawson E.E., and Knowles M.R. Decreased sodiumion absorption across nasal epithelium of very premature infants with respiratory distress syndrome. J. Pediatr. 130 (1997) 373-377
    • (1997) J. Pediatr. , vol.130 , pp. 373-377
    • Barker, P.M.1    Gowen, C.W.2    Lawson, E.E.3    Knowles, M.R.4
  • 16
    • 0017664223 scopus 로고
    • Physics of chemoreception
    • Berg H.C., and Purcell E.M. Physics of chemoreception. Biophys. J. 20 (1977) 193-219
    • (1977) Biophys. J. , vol.20 , pp. 193-219
    • Berg, H.C.1    Purcell, E.M.2
  • 17
    • 0000909389 scopus 로고
    • Functions of CFTR other than as a plasma membrane chloride channel.
    • Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds), Wiley, Chichester
    • Biwersi J., and Verkman A.S. Functions of CFTR other than as a plasma membrane chloride channel. In: Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds). "Cystic Fibrosis-Current Topics. 2 (1994), Wiley, Chichester 155-171
    • (1994) "Cystic Fibrosis-Current Topics. , vol.2 , pp. 155-171
    • Biwersi, J.1    Verkman, A.S.2
  • 18
    • 0029861046 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator activation stimulates endosome fusion in vivo
    • Biwersi J., Emans N., and Verkman A.S. Cystic fibrosis transmembrane conductance regulator activation stimulates endosome fusion in vivo. Natl. Acad. Scio. U.S.A. 93 (1996) 12484-12489
    • (1996) Natl. Acad. Scio. U.S.A. , vol.93 , pp. 12484-12489
    • Biwersi, J.1    Emans, N.2    Verkman, A.S.3
  • 19
    • 0030929698 scopus 로고    scopus 로고
    • Cystic fibrosis and non-cystic fibrosis human nasal epithelium show analogous Na absorbtion and reversible block by phenamil
    • Blank U., Rückes C., Clauss W., Hofmann T., Lindeman H., Munker G., and Weber W.M. Cystic fibrosis and non-cystic fibrosis human nasal epithelium show analogous Na absorbtion and reversible block by phenamil. Pfluegers Arch. 434 (1997) 19-24
    • (1997) Pfluegers Arch. , vol.434 , pp. 19-24
    • Blank, U.1    Rückes, C.2    Clauss, W.3    Hofmann, T.4    Lindeman, H.5    Munker, G.6    Weber, W.M.7
  • 20
  • 21
  • 22
    • 0018841950 scopus 로고
    • Airway transepithelial electric potential in vivo: Species and regional differences.
    • Boucher Bromberg R.C.P.A., and Gatzy J.T. Airway transepithelial electric potential in vivo: Species and regional differences. J. Appl. Physiol. 48 (1980) 169-176
    • (1980) J. Appl. Physiol. , vol.48 , pp. 169-176
    • Boucher Bromberg, R.C.P.A.1    Gatzy, J.T.2
  • 23
    • 0019387331 scopus 로고
    • Regional differences in bioelectric properties and ion flow in excised canine airways
    • Boucher Stutts R.C.M.J., and Gatzy J.T. Regional differences in bioelectric properties and ion flow in excised canine airways. J. Appl. Physiol. 51 (1981) 706-714
    • (1981) J. Appl. Physiol. , vol.51 , pp. 706-714
    • Boucher Stutts, R.C.M.J.1    Gatzy, J.T.2
  • 24
    • 0022973473 scopus 로고
    • + transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation
    • + transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J. Clin. Invest. 78 (1986) 1245-1252
    • (1986) J. Clin. Invest. , vol.78 , pp. 1245-1252
    • Boucher Stutts, R.C.1    Knowles, M.J.M.R.2    Cantley, L.3    Gatzy, J.T.4
  • 29
    • 0031921269 scopus 로고    scopus 로고
    • + channel (ENaCs) in Xenopus oocytes co-expressing CFTR and ENaC
    • + channel (ENaCs) in Xenopus oocytes co-expressing CFTR and ENaC. J. Physiol. (London) 508 (1998) 825-836
    • (1998) J. Physiol. (London) , vol.508 , pp. 825-836
    • Briel, M.1    Greger, R.2    Kunzelmann, K.3
  • 31
    • 0027483065 scopus 로고
    • Epithelial sodium channel related to proteins involved in neurodegeneration
    • Canessa C.M., Horisberger J.-D., and Rossier B.C. Epithelial sodium channel related to proteins involved in neurodegeneration. Nature (London) 361 (1993) 467-470
    • (1993) Nature (London) , vol.361 , pp. 467-470
    • Canessa, C.M.1    Horisberger, J.-D.2    Rossier, B.C.3
  • 33
    • 0029102078 scopus 로고
    • + channel regulation
    • + channel regulation. Kidney Int. 48 (1995) 970-984
    • (1995) Kidney Int. , vol.48 , pp. 970-984
    • Cantiello, H.F.1
  • 35
    • 0023767373 scopus 로고
    • Functional reconstitution of an isolated sodium channel from bovine trachea
    • Cherksey B.D. Functional reconstitution of an isolated sodium channel from bovine trachea. Comp. Biochem. Physiol. A 90 (1988) 771-773
    • (1988) Comp. Biochem. Physiol. A , vol.90 , pp. 771-773
    • Cherksey, B.D.1
  • 36
    • 0018797528 scopus 로고
    • Rotational and lateral diffusion of membrane proteins
    • Cherry R.J. Rotational and lateral diffusion of membrane proteins. Biochim. Biophys. Acta 559 (1979) 289-327
    • (1979) Biochim. Biophys. Acta , vol.559 , pp. 289-327
    • Cherry, R.J.1
  • 38
    • 0028276637 scopus 로고
    • Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: A patch-clamp study
    • Chinet Fullton T.C., Yankaskas J.M.J.R., Boucher R.C., and Stutts M.J. Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: A patch-clamp study. Am. J. Physiol. 266 (1994) C1061-C1068
    • (1994) Am. J. Physiol. , vol.266
    • Chinet Fullton, T.C.1    Yankaskas, J.M.J.R.2    Boucher, R.C.3    Stutts, M.J.4
  • 39
    • 0026523829 scopus 로고
    • Cystic fibrosis: Molecular biology and therapeutic implications
    • Collins F.S. Cystic fibrosis: Molecular biology and therapeutic implications. Science 256 (1992) 774-779
    • (1992) Science , vol.256 , pp. 774-779
    • Collins, F.S.1
  • 40
    • 0021059582 scopus 로고
    • Bioelectric properties and ion transport of airways excised from adult and fetal sheep
    • Cotton C.U., Lawson E.E., Boucher R.C., and Gatzy J.T. Bioelectric properties and ion transport of airways excised from adult and fetal sheep. J. Appl. Physiol. 55 (1983) 1542-1549
    • (1983) J. Appl. Physiol. , vol.55 , pp. 1542-1549
    • Cotton, C.U.1    Lawson, E.E.2    Boucher, R.C.3    Gatzy, J.T.4
  • 41
    • 0023150501 scopus 로고
    • Abnormal apical cell membrane in cystic fibrosis respiratory epithelium. An in vitro electrophysiologic analysis
    • Cotton C.U., Stutts M.J., Knowles M.R., Gatzy J.T., and Boucher R.C. Abnormal apical cell membrane in cystic fibrosis respiratory epithelium. An in vitro electrophysiologic analysis. J. Clin. Invest. 79 (1987) 80-85
    • (1987) J. Clin. Invest. , vol.79 , pp. 80-85
    • Cotton, C.U.1    Stutts, M.J.2    Knowles, M.R.3    Gatzy, J.T.4    Boucher, R.C.5
  • 42
    • 0024266438 scopus 로고
    • Bioelectric properties and ion transport across excised canine fetal and neonatal airways
    • Cotton C.U., Boucher R.C., and Gatzy J.T. Bioelectric properties and ion transport across excised canine fetal and neonatal airways. J. Appl. Physiol. 65 (1988) 2367-2375
    • (1988) J. Appl. Physiol. , vol.65 , pp. 2367-2375
    • Cotton, C.U.1    Boucher, R.C.2    Gatzy, J.T.3
  • 43
    • 0028220333 scopus 로고
    • Heterogeneity in the severity of cystic fibrosis and the role of CFTR gene mutations
    • Dean M., and Santis G. Heterogeneity in the severity of cystic fibrosis and the role of CFTR gene mutations. Hum. Genet. 93 (1994) 364-368
    • (1994) Hum. Genet. , vol.93 , pp. 364-368
    • Dean, M.1    Santis, G.2
  • 44
    • 0025312731 scopus 로고
    • Multiple mutations in highly conserved residues are found in mildly affected cystic fibrosis patients
    • Dean M., White M.B., Amos J., Gerrard B., Stewart Khaw C.K.T., and Leppert M. Multiple mutations in highly conserved residues are found in mildly affected cystic fibrosis patients. Cell (Cambridge, Mass.) 61 (1990) 863-870
    • (1990) Cell (Cambridge, Mass.) , vol.61 , pp. 863-870
    • Dean, M.1    White, M.B.2    Amos, J.3    Gerrard, B.4    Stewart Khaw, C.K.T.5    Leppert, M.6
  • 45
    • 0030956949 scopus 로고    scopus 로고
    • Nasal potential difference in cystic fibrosis patients presenting borderline sweat test
    • Delmarco A., Pradal U., Cabrini G., Bonizzato A., and Mastella G. Nasal potential difference in cystic fibrosis patients presenting borderline sweat test. Eur. Respir. J. 10 (1997) 1145-1149
    • (1997) Eur. Respir. J. , vol.10 , pp. 1145-1149
    • Delmarco, A.1    Pradal, U.2    Cabrini, G.3    Bonizzato, A.4    Mastella, G.5
  • 47
    • 0024786312 scopus 로고
    • Cystic fibrosis affects chloride and sodium channels in human airway epithelia
    • Duszyk French M.A.S., and Man S.F. Cystic fibrosis affects chloride and sodium channels in human airway epithelia. Can. J. Physiol. Pharmacol. 67 (1989) 1362-1365
    • (1989) Can. J. Physiol. Pharmacol. , vol.67 , pp. 1362-1365
    • Duszyk French, M.A.S.1    Man, S.F.2
  • 48
    • 0345941706 scopus 로고
    • Rotational and lateral diffusion of membrane proteins and lipids: Phenomena and function
    • Edidin M. Rotational and lateral diffusion of membrane proteins and lipids: Phenomena and function. Curr. Top. Membr. Transp. 29 (1987) 91-127
    • (1987) Curr. Top. Membr. Transp. , vol.29 , pp. 91-127
    • Edidin, M.1
  • 49
    • 0017819582 scopus 로고
    • Lateral diffusion in phospholipid bilayer membranes and multilamellar liquid crystals
    • Fahey P.F., and Webb W.W. Lateral diffusion in phospholipid bilayer membranes and multilamellar liquid crystals. Biochemistry 17 (1978) 3046-3053
    • (1978) Biochemistry , vol.17 , pp. 3046-3053
    • Fahey, P.F.1    Webb, W.W.2
  • 51
    • 0027670557 scopus 로고
    • Identification of nonselective cation channels in cultured adult rat alveolar type II cells
    • Feng Z.P., Clark R.B., and Berthiaume Y. Identification of nonselective cation channels in cultured adult rat alveolar type II cells. Am. J. Respir. Cell. Mol. Biol. 9 (1993) 248-254
    • (1993) Am. J. Respir. Cell. Mol. Biol. , vol.9 , pp. 248-254
    • Feng, Z.P.1    Clark, R.B.2    Berthiaume, Y.3
  • 52
    • 0029810356 scopus 로고    scopus 로고
    • Genotype-phenotype correlation in cystic fibrosis patients
    • Ferrari M., and Cremonesi L. Genotype-phenotype correlation in cystic fibrosis patients. Ann. Biol. Clin. (Paris) 54 (1996) 235-241
    • (1996) Ann. Biol. Clin. (Paris) , vol.54 , pp. 235-241
    • Ferrari, M.1    Cremonesi, L.2
  • 53
    • 0030451774 scopus 로고    scopus 로고
    • Cell surface expression of the epithelial Na channel and a mutant causing Liddle syndrome: A quantitative approach
    • Firsov D., Schild L., Gautschi I., Merillat A.M., Schneeberger E., and Rossier B.C. Cell surface expression of the epithelial Na channel and a mutant causing Liddle syndrome: A quantitative approach. Proc. Natl. Acad. Set. U.S.A. 93 (1996) 15370-15375
    • (1996) Proc. Natl. Acad. Set. U.S.A. , vol.93 , pp. 15370-15375
    • Firsov, D.1    Schild, L.2    Gautschi, I.3    Merillat, A.M.4    Schneeberger, E.5    Rossier, B.C.6
  • 54
    • 0032518665 scopus 로고    scopus 로고
    • The heterotetrameric architecture of the epithelial sodium channel (ENaC)
    • Firsov D., Gautschi I., Merillat A.M., Rossier B.C., and Schild L. The heterotetrameric architecture of the epithelial sodium channel (ENaC). EMBO J. 17 (1998) 344-352
    • (1998) EMBO J. , vol.17 , pp. 344-352
    • Firsov, D.1    Gautschi, I.2    Merillat, A.M.3    Rossier, B.C.4    Schild, L.5
  • 55
    • 0028940005 scopus 로고
    • Functions of the cystic fibrosis transmembrane conductance regulator protein
    • Frizzell R.A. Functions of the cystic fibrosis transmembrane conductance regulator protein. Am. J. Respir. Crit. Care Med. 151 (1995) S54-S58
    • (1995) Am. J. Respir. Crit. Care Med. , vol.151
    • Frizzell, R.A.1
  • 57
    • 0025096102 scopus 로고
    • Aerosolized amiloride for the treatment of lung disease in cystic fibrosis
    • (Letter to the Editor)
    • Gallo R.L. Aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N. Engl. J. Med. 323 (1990) 996-997 (Letter to the Editor)
    • (1990) N. Engl. J. Med. , vol.323 , pp. 996-997
    • Gallo, R.L.1
  • 58
    • 0030945484 scopus 로고    scopus 로고
    • Epithelial sodium channels: Function, structure and regulation
    • Garty H., and Palmer L.G. Epithelial sodium channels: Function, structure and regulation. Physiol. Rev. 77 (1997) 359-396
    • (1997) Physiol. Rev. , vol.77 , pp. 359-396
    • Garty, H.1    Palmer, L.G.2
  • 61
    • 0002089547 scopus 로고
    • Genotype/phenotype relationships in cystic fibrosis.
    • Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds), Wiley, Chichester
    • Hamosh A., and Cutting G.R. Genotype/phenotype relationships in cystic fibrosis. In: Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds). "Cystic Fibrosis-Current Topics. 1 (1993), Wiley, Chichester 69-89
    • (1993) "Cystic Fibrosis-Current Topics. , vol.1 , pp. 69-89
    • Hamosh, A.1    Cutting, G.R.2
  • 64
    • 0029586683 scopus 로고
    • A de novo missense mutation of the β subunit of the epithelial sodium channel causes hypertension and Liddle syndrome, identifying a proline-rich segment critical for regulation of channel activity
    • Hansson Schild J.H., Lu L.Y., Wilson T., Gautschi I., Shimkets R., Nelson-Williams Rossier C.B., and Lifton R. A de novo missense mutation of the β subunit of the epithelial sodium channel causes hypertension and Liddle syndrome, identifying a proline-rich segment critical for regulation of channel activity. Proc. Natl. Acad. Scio. U.S.A. 92 (1995) 11495-11499
    • (1995) Proc. Natl. Acad. Scio. U.S.A. , vol.92 , pp. 11495-11499
    • Hansson Schild, J.H.1    Lu, L.Y.2    Wilson, T.3    Gautschi, I.4    Shimkets, R.5    Nelson-Williams Rossier, C.B.6    Lifton, R.7
  • 65
    • 0021808414 scopus 로고
    • Transepithelial potential difference in cystic fibrosis
    • Hay J.G., and Geddes D.M. Transepithelial potential difference in cystic fibrosis. Thorax 40 (1985) 493-496
    • (1985) Thorax , vol.40 , pp. 493-496
    • Hay, J.G.1    Geddes, D.M.2
  • 66
    • 0028802876 scopus 로고
    • Modification of nasal epithelial potential differences of individuals with cystic fibrosis consequent to local administration of a normal CFTR cDNA adenovirus gene transfer vector
    • Hay J.G., McElvaney N.G., Herena J., and Crystal R.G. Modification of nasal epithelial potential differences of individuals with cystic fibrosis consequent to local administration of a normal CFTR cDNA adenovirus gene transfer vector. Hum. Gene Ther. 6 (1995) 1487-1496
    • (1995) Hum. Gene Ther. , vol.6 , pp. 1487-1496
    • Hay, J.G.1    McElvaney, N.G.2    Herena, J.3    Crystal, R.G.4
  • 67
    • 0026471963 scopus 로고
    • Cystic fibrosis transmembrane conductance regulator (CFTR)
    • Higgins C.F. Cystic fibrosis transmembrane conductance regulator (CFTR). Br. Med. Bull. 48 (1992) 754-765
    • (1992) Br. Med. Bull. , vol.48 , pp. 754-765
    • Higgins, C.F.1
  • 69
    • 0030924036 scopus 로고    scopus 로고
    • Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis
    • Ho L.P., Samways J.M., Porteous D.J., Dorin J.R., Carothers A., Greening A.P., and Innes J.A. Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis. Eur. Respir. J. 10 (1997) 2018-2022
    • (1997) Eur. Respir. J. , vol.10 , pp. 2018-2022
    • Ho, L.P.1    Samways, J.M.2    Porteous, D.J.3    Dorin, J.R.4    Carothers, A.5    Greening, A.P.6    Innes, J.A.7
  • 75
    • 0030893188 scopus 로고    scopus 로고
    • The biogenesis, traffic, and function of the cystic fibrosis transmembrane conductance regulator
    • Jilling T., and Kirk K.L. The biogenesis, traffic, and function of the cystic fibrosis transmembrane conductance regulator. Int. Rev. Cytol. 172 (1997) 193-241
    • (1997) Int. Rev. Cytol. , vol.172 , pp. 193-241
    • Jilling, T.1    Kirk, K.L.2
  • 76
    • 0025271764 scopus 로고
    • Identification of a voltage- and calcium-dependent non-selective cation channel in cultured adult and fetal human nasal epithelial cells
    • Jorissen M., Vereecke J., Carmeliet E., Van den Berghe H., and Cassiman J.J. Identification of a voltage- and calcium-dependent non-selective cation channel in cultured adult and fetal human nasal epithelial cells. Pfluegers Arch. 415 (1990) 617-623
    • (1990) Pfluegers Arch. , vol.415 , pp. 617-623
    • Jorissen, M.1    Vereecke, J.2    Carmeliet, E.3    Van den Berghe, H.4    Cassiman, J.J.5
  • 77
    • 0028899752 scopus 로고
    • Cystic fibrosis transmembrane conductance regulator is required for protein kinase A activation of an outwardly rectified anion channel purified from bovine tracheal epithelia
    • Jovov B., Ismailov I.I., and Benos D.J. Cystic fibrosis transmembrane conductance regulator is required for protein kinase A activation of an outwardly rectified anion channel purified from bovine tracheal epithelia. J. Biol. Chem. 270 (1995) 1521-1528
    • (1995) J. Biol. Chem. , vol.270 , pp. 1521-1528
    • Jovov, B.1    Ismailov, I.I.2    Benos, D.J.3
  • 79
    • 0019490443 scopus 로고
    • Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo
    • Knowles M.R., Carson J.L., Collier A.M., Gatzy J.T., and Boucher R.C. Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo. Am. Rev. Respire. Dis. 124 (1981) 484-490
    • (1981) Am. Rev. Respire. Dis. , vol.124 , pp. 484-490
    • Knowles, M.R.1    Carson, J.L.2    Collier, A.M.3    Gatzy, J.T.4    Boucher, R.C.5
  • 80
    • 0019809960 scopus 로고
    • Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis
    • Knowles M., Gatzy J., and Boucher R. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N. Engl. J. Med. 305 (1981) 1489-1495
    • (1981) N. Engl. J. Med. , vol.305 , pp. 1489-1495
    • Knowles, M.1    Gatzy, J.2    Boucher, R.3
  • 81
    • 0019985202 scopus 로고
    • Measurements of transepithelial electric potential differences in the trachea and bronchi of human subjects in vivo
    • Knowles M.R., Buntin W.H., Bromberg P.A., Gatzy J.T., and Boucher R.C. Measurements of transepithelial electric potential differences in the trachea and bronchi of human subjects in vivo. Am. Rev. Respire. Dis. 126 (1982) 108-112
    • (1982) Am. Rev. Respire. Dis. , vol.126 , pp. 108-112
    • Knowles, M.R.1    Buntin, W.H.2    Bromberg, P.A.3    Gatzy, J.T.4    Boucher, R.C.5
  • 82
    • 0020596438 scopus 로고
    • Relative ion permeability of normal and cystic fibrosis nasal epithelium
    • Knowles M., Gatzy J., and Boucher R. Relative ion permeability of normal and cystic fibrosis nasal epithelium. J. Clin. Invest. 71 (1983) 1410-1417
    • (1983) J. Clin. Invest. , vol.71 , pp. 1410-1417
    • Knowles, M.1    Gatzy, J.2    Boucher, R.3
  • 87
    • 0000930452 scopus 로고
    • Amiloride in cystic fibrosis: Safety, pharmacokinetics, and efficacy in the treatment of pulmonary disease.
    • Cragoe Jr. E.J., Kleyman T.R., and Simchowitz L. (Eds), VCH Publishers, New York
    • Knowles M.R., Church N.L., Waltner W.E., Gatzy J.T., and Boucher R.C. Amiloride in cystic fibrosis: Safety, pharmacokinetics, and efficacy in the treatment of pulmonary disease. In: Cragoe Jr. E.J., Kleyman T.R., and Simchowitz L. (Eds). "Amiloride and Its Analogs: Unique Cation Transport Inhibitors" (1992), VCH Publishers, New York 301-316
    • (1992) "Amiloride and Its Analogs: Unique Cation Transport Inhibitors" , pp. 301-316
    • Knowles, M.R.1    Church, N.L.2    Waltner, W.E.3    Gatzy, J.T.4    Boucher, R.C.5
  • 88
    • 0028991826 scopus 로고
    • Vivo nasal potential difference: Techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis
    • Knowles M.R., Paradiso A.M., and Boucher R.C. Vivo nasal potential difference: Techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. Hum. Gene Ther. 6 (1995) 445-455
    • (1995) Hum. Gene Ther. , vol.6 , pp. 445-455
    • Knowles, M.R.1    Paradiso, A.M.2    Boucher, R.C.3
  • 89
    • 0028929841 scopus 로고
    • Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis
    • Knowles M.R., Olivier K., Noone P., and Boucher R.C. Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis. Am. J. Respir. Crit. Care Med. 151 (1995) S65-S69
    • (1995) Am. J. Respir. Crit. Care Med. , vol.151
    • Knowles, M.R.1    Olivier, K.2    Noone, P.3    Boucher, R.C.4
  • 91
    • 0022988338 scopus 로고
    • Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses
    • Kohler D., App E., Schmitz-Schumann M., Wurtemberger G., and Matthys H. Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses. Eur. J. Respir. Dis., Suppl. 146 (1986) 319-326
    • (1986) Eur. J. Respir. Dis., Suppl. , vol.146 , pp. 319-326
    • Kohler, D.1    App, E.2    Schmitz-Schumann, M.3    Wurtemberger, G.4    Matthys, H.5
  • 95
    • 0031024767 scopus 로고    scopus 로고
    • + currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator
    • + currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator. FEBS Lett. 400 (1997) 341-344
    • (1997) FEBS Lett. , vol.400 , pp. 341-344
    • Kunzelmann, K.1    Kiser, G.L.2    Schreiber, R.3    Riordan, J.R.4
  • 97
    • 0030068024 scopus 로고    scopus 로고
    • Molecular genetics of human blood pressure variation
    • Lifton R.P. Molecular genetics of human blood pressure variation. Science 272 (1996) 676-680
    • (1996) Science , vol.272 , pp. 676-680
    • Lifton, R.P.1
  • 102
    • 0032127980 scopus 로고    scopus 로고
    • + conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways
    • + conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways. J. Clin. Invest. 102 (1998) 15-21
    • (1998) J. Clin. Invest. , vol.102 , pp. 15-21
    • Mall, M.1    Briel, M.2    Greger, R.3    Schreiber, R.4    Kunzelmann, K.5
  • 103
    • 0029792652 scopus 로고    scopus 로고
    • Localization of epithelial sodium channel subunit mRNAs in adult rat lung by in situ hybridization
    • Matsushita K., McCray Jr. P.B., Sigmund R.D., Welsh M.J., and Stokes J.B. Localization of epithelial sodium channel subunit mRNAs in adult rat lung by in situ hybridization. Am. J. Physiol. 271 (1996) L332-L339
    • (1996) Am. J. Physiol. , vol.271
    • Matsushita, K.1    McCray Jr., P.B.2    Sigmund, R.D.3    Welsh, M.J.4    Stokes, J.B.5
  • 104
    • 0000260746 scopus 로고
    • Cystic fibrosis: The first 50 years. A review of the clinical problems and their management.
    • Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds), Wiley, Chichester
    • Mearns M.B. Cystic fibrosis: The first 50 years. A review of the clinical problems and their management. In: Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds). "Cystic Fibrosis-Current Topics. 1 (1993), Wiley, Chichester 217-250
    • (1993) "Cystic Fibrosis-Current Topics. , vol.1 , pp. 217-250
    • Mearns, M.B.1
  • 105
    • 0027216541 scopus 로고
    • Effect of amiloride and saline on nasal mucociliary clearance and potential difference in cystic fibrosis and normal subjects
    • Middleton P.G., Geddes D.M., and Alton E.W. Effect of amiloride and saline on nasal mucociliary clearance and potential difference in cystic fibrosis and normal subjects. Thorax 48 (1993) 812-816
    • (1993) Thorax , vol.48 , pp. 812-816
    • Middleton, P.G.1    Geddes, D.M.2    Alton, E.W.3
  • 106
    • 0028020058 scopus 로고
    • Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium
    • Middleton P.G., Geddes D.M., and Alton E.W. Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium. Eur. Respir. J. 7 (1994) 2050-2056
    • (1994) Eur. Respir. J. , vol.7 , pp. 2050-2056
    • Middleton, P.G.1    Geddes, D.M.2    Alton, E.W.3
  • 109
    • 0012050693 scopus 로고
    • Lateral and rotational diffusion of bacteriorodopsin in lipid bilayers: Experimental test of the Saffman-Delbruck equations
    • Peters R., and Cherry R.J. Lateral and rotational diffusion of bacteriorodopsin in lipid bilayers: Experimental test of the Saffman-Delbruck equations. Proc. Nat. Acad. Sci. U.S.A. 79 (1982) 4317-4321
    • (1982) Proc. Nat. Acad. Sci. U.S.A. , vol.79 , pp. 4317-4321
    • Peters, R.1    Cherry, R.J.2
  • 111
    • 0025349031 scopus 로고
    • Cystic fibrosis: A disease in electrolyte transport
    • Quinton P.M. Cystic fibrosis: A disease in electrolyte transport. FASEB J. 4 (1990) 2709-2717
    • (1990) FASEB J. , vol.4 , pp. 2709-2717
    • Quinton, P.M.1
  • 112
    • 0020533435 scopus 로고
    • Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis
    • Quinton P.M., and Bijman J. Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis. N. Engl. J. Med. 308 (1983) 1185-1189
    • (1983) N. Engl. J. Med. , vol.308 , pp. 1185-1189
    • Quinton, P.M.1    Bijman, J.2
  • 114
    • 0027481813 scopus 로고
    • The cystic fibrosis transmembrane conductance regulator
    • Riordan J.R. The cystic fibrosis transmembrane conductance regulator. Annu. Rev. Physiol. 55 (1993) 609-630
    • (1993) Annu. Rev. Physiol. , vol.55 , pp. 609-630
    • Riordan, J.R.1
  • 117
    • 0023641255 scopus 로고
    • Clinical application of transepithelial potential difference measurements in cystic fibrosis
    • Sauder R.A., Chesrown S.E., and Loughlin G.M. Clinical application of transepithelial potential difference measurements in cystic fibrosis. J. Pediatr. 111 (1987) 353-358
    • (1987) J. Pediatr. , vol.111 , pp. 353-358
    • Sauder, R.A.1    Chesrown, S.E.2    Loughlin, G.M.3
  • 121
    • 0028810344 scopus 로고
    • Effect of vasopressin and aldosterone on the lateral mobility of epithelial Na channels in A6 renal epithelial cells
    • Smith P.R., Stoner Viggiano L.C., Angelides S.C.K.J., and Benos D.J. Effect of vasopressin and aldosterone on the lateral mobility of epithelial Na channels in A6 renal epithelial cells. J. Membr. Biol. 147 (1995) 195-205
    • (1995) J. Membr. Biol. , vol.147 , pp. 195-205
    • Smith, P.R.1    Stoner Viggiano, L.C.2    Angelides, S.C.K.J.3    Benos, D.J.4
  • 123
    • 0031985150 scopus 로고    scopus 로고
    • Electrophysiological and biochemical evidence that DEG/ENaC cation channels are composed of nine subunits
    • Snyder P.M., Cheng Prince C., Rogers L.S.J.C., and Welsh M.J. Electrophysiological and biochemical evidence that DEG/ENaC cation channels are composed of nine subunits. J. Biol. Chem. 273 (1998) 681-684
    • (1998) J. Biol. Chem. , vol.273 , pp. 681-684
    • Snyder, P.M.1    Cheng Prince, C.2    Rogers, L.S.J.C.3    Welsh, M.J.4
  • 125
    • 0030970422 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator inverts protein kinase A- mediated regulation of epithelial sodium channel single channel kinetics
    • Stutts M.J., Rossier B.C., and Boucher R.C. Cystic fibrosis transmembrane conductance regulator inverts protein kinase A- mediated regulation of epithelial sodium channel single channel kinetics. J. Biol. Chem. 272 (1997) 14037-14040
    • (1997) J. Biol. Chem. , vol.272 , pp. 14037-14040
    • Stutts, M.J.1    Rossier, B.C.2    Boucher, R.C.3
  • 126
    • 0028969478 scopus 로고
    • Insulin-activated amiloride-blockable nonselective cation and NaH channels in the fetal distal lung epithelium
    • Tohda H., and Marunaka Y. Insulin-activated amiloride-blockable nonselective cation and NaH channels in the fetal distal lung epithelium. Gen. Pharmacol. 26 (1995) 755-763
    • (1995) Gen. Pharmacol. , vol.26 , pp. 755-763
    • Tohda, H.1    Marunaka, Y.2
  • 128
    • 0025937887 scopus 로고
    • Biochemical and molecular genetics of cystic fibrosis
    • Tsui L.-C., and Buchwald M. Biochemical and molecular genetics of cystic fibrosis. Adv. Hum. Genet. 20 (1991) 153-166
    • (1991) Adv. Hum. Genet. , vol.20 , pp. 153-166
    • Tsui, L.-C.1    Buchwald, M.2
  • 129
    • 0010484763 scopus 로고
    • Mutation analysis in cystic fibrosis.
    • Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds), Wiley, Chichester
    • Tsui L.-C., Markiewics D., Zielenski J., Corey M., and Durie P. Mutation analysis in cystic fibrosis. In: Dodge J.A., Brock D.J.H., and Widdicombe J.H. (Eds). "Cystic Fibrosis-Current Topics. 1 (1993), Wiley, Chichester 27-44
    • (1993) "Cystic Fibrosis-Current Topics. , vol.1 , pp. 27-44
    • Tsui, L.-C.1    Markiewics, D.2    Zielenski, J.3    Corey, M.4    Durie, P.5
  • 131
    • 0027162649 scopus 로고
    • Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
    • Welsh M.J., and Smith A.E. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell (Cambridge, Mass.) 73 (1993) 1251-1254
    • (1993) Cell (Cambridge, Mass.) , vol.73 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 132
    • 0019064701 scopus 로고
    • Anion selectivity of the chloride-transport process in dog tracheal epithelium
    • Widdicombe J.H., and Welsh M.J. Anion selectivity of the chloride-transport process in dog tracheal epithelium. Am. J. Physiol. 239 (1980) C112-C117
    • (1980) Am. J. Physiol. , vol.239
    • Widdicombe, J.H.1    Welsh, M.J.2
  • 133
    • 0025997759 scopus 로고
    • Sodium transport and intracellular sodium activity in cultured human nasal epithelium
    • Willumsen N.J., and Boucher R.C. Sodium transport and intracellular sodium activity in cultured human nasal epithelium. Am. J. Physiol. 261 (1991) C319-C331
    • (1991) Am. J. Physiol. , vol.261
    • Willumsen, N.J.1    Boucher, R.C.2
  • 134
    • 0025995248 scopus 로고
    • Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium
    • Willumsen N.J., and Boucher R.C. Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium. Am. J. Physiol. 261 (1991) C332-C341
    • (1991) Am. J. Physiol. , vol.261
    • Willumsen, N.J.1    Boucher, R.C.2
  • 135
    • 0026636673 scopus 로고
    • Differentiated structure and function of cultures from human tracheal epithelium
    • Yamaya M., Finkbeiner W.E., Chun S.Y., and Widdicombe J.H. Differentiated structure and function of cultures from human tracheal epithelium. Am. J. Physiol. 262 (1992) L713-L724
    • (1992) Am. J. Physiol. , vol.262
    • Yamaya, M.1    Finkbeiner, W.E.2    Chun, S.Y.3    Widdicombe, J.H.4
  • 136
    • 0022396481 scopus 로고
    • Culture of human nasal epithelial cells on collagen matrix supports. A comparison of bioelectric properties of normal and cystic fibrosis epithelia
    • Yankaskas J.R., Cotton C.U., Knowles M.R., Gatzy J.T., and Boucher R.C. Culture of human nasal epithelial cells on collagen matrix supports. A comparison of bioelectric properties of normal and cystic fibrosis epithelia. Am. Rev. Respir. Dis. 132 (1985) 1281-1287
    • (1985) Am. Rev. Respir. Dis. , vol.132 , pp. 1281-1287
    • Yankaskas, J.R.1    Cotton, C.U.2    Knowles, M.R.3    Gatzy, J.T.4    Boucher, R.C.5
  • 137
    • 0021981520 scopus 로고
    • Persistence of abnormal chloride ion permeability in cystic fibrosis nasal epithelial cells in heterologous culture
    • Yankaskas J.R., Knowles M.R., Gatzy J.T., and Boucher R.C. Persistence of abnormal chloride ion permeability in cystic fibrosis nasal epithelial cells in heterologous culture. Lancet 1 (1985) 954-956
    • (1985) Lancet , vol.1 , pp. 954-956
    • Yankaskas, J.R.1    Knowles, M.R.2    Gatzy, J.T.3    Boucher, R.C.4
  • 140
    • 0028028636 scopus 로고
    • Regulation of low-amiloride-affinity sodium channels in alveolar type II cells
    • Yue G., Shoemaker R.L., and Matalon S. Regulation of low-amiloride-affinity sodium channels in alveolar type II cells. Am. J. Physiol. 267 (1994) L94-L100
    • (1994) Am. J. Physiol. , vol.267
    • Yue, G.1    Shoemaker, R.L.2    Matalon, S.3


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