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Volumn 104, Issue 6, 1998, Pages 576-590

Cystic fibrosis: A multiple exocrinopathy caused by dysfunctions in a multifunctional transport protein

Author keywords

[No Author keywords available]

Indexed keywords

ABC TRANSPORTER; CARRIER PROTEIN; CHLORIDE CHANNEL; DEFENSIN; DORNASE ALFA; GELSOLIN; IBUPROFEN; RECOMBINANT PROTEIN; REGULATOR PROTEIN; TRANSMEMBRANE CONDUCTANCE REGULATOR;

EID: 0032104299     PISSN: 00029343     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0002-9343(98)00119-3     Document Type: Article
Times cited : (37)

References (123)
  • 1
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
    • 1. Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989;245:1066-1073.
    • (1989) Science , vol.245 , pp. 1066-1073
    • Riordan, J.R.1    Rommens, J.M.2    Kerem, B.3
  • 2
    • 0024453308 scopus 로고
    • Identification of the cystic fibrosis gene: Chromosome walking and jumping
    • 2. Rommens JM, Iannuzzi MC, Kerem B-S, et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science. 1989;245:1059-1065.
    • (1989) Science , vol.245 , pp. 1059-1065
    • Rommens, J.M.1    Iannuzzi, M.C.2    Kerem, B.-S.3
  • 3
    • 0027162649 scopus 로고
    • Molecular mechanisms of CFTR chloride channel dysfunction in CF
    • 3. Welsh MJ, Smith AE. Molecular mechanisms of CFTR chloride channel dysfunction in CF. Cell. 1993;73:1251-1254.
    • (1993) Cell , vol.73 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 4
    • 0026523829 scopus 로고
    • Cystic fibrosis: Molecular biology and therapeutic implications
    • 4. Collins FS. Cystic fibrosis: molecular biology and therapeutic implications. Science. 1992;256:774-779.
    • (1992) Science , vol.256 , pp. 774-779
    • Collins, F.S.1
  • 5
    • 0023472728 scopus 로고
    • Electrolyte transport by airway epithelia
    • 5. Welsh MJ. Electrolyte transport by airway epithelia. Physiol Rev. 1987;67:1143-1183.
    • (1987) Physiol Rev. , vol.67 , pp. 1143-1183
    • Welsh, M.J.1
  • 6
    • 0026046164 scopus 로고
    • Epithelial ion transport in the fetal and perinatal lung
    • 6. O'Brodovich H. Epithelial ion transport in the fetal and perinatal lung. Am J Physiol (Cell Physiol). 1991;261:C555-C564.
    • (1991) Am J Physiol (Cell Physiol) , vol.261
    • O'Brodovich, H.1
  • 7
    • 0029934215 scopus 로고    scopus 로고
    • Management of pulmonary disease in patients with cystic fibrosis
    • 7. Ramsey BW. Management of pulmonary disease in patients with cystic fibrosis. NEJM. 1996;335:179-188.
    • (1996) NEJM , vol.335 , pp. 179-188
    • Ramsey, B.W.1
  • 8
    • 0029164287 scopus 로고
    • The ABC of channel regulation
    • 8. Higgins CF. The ABC of channel regulation. Cell. 1995;82:693-696.
    • (1995) Cell , vol.82 , pp. 693-696
    • Higgins, C.F.1
  • 9
    • 0026621245 scopus 로고
    • ABC transporters: From microorganisms to man
    • 9. Higgins CF. ABC transporters: from microorganisms to man. Annu Rev Cell Biol. 1992;8:67-113.
    • (1992) Annu Rev Cell Biol. , vol.8 , pp. 67-113
    • Higgins, C.F.1
  • 12
    • 0007544439 scopus 로고    scopus 로고
    • mdr1 P-glycoprotein is a lipid translocase of broad specificity, while mdr3 P-glycoprotein specifically translocates phosphatidylcholine
    • 12. 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;87:507-517.
    • (1996) Cell , vol.87 , pp. 507-517
    • Van Helvoort, A.1    Smith, A.J.2    Sprong, H.3
  • 13
    • 0029985152 scopus 로고    scopus 로고
    • 4 transport by co-expression of both half molecules of human multidrug resistance protein in insect cells
    • 4 transport by co-expression of both half molecules of human multidrug resistance protein in insect cells. J Biol Ghent. 1996;271:27782-27787.
    • (1996) J Biol Ghent. , vol.271 , pp. 27782-27787
    • Gao, M.1    Loe, D.W.2    Grant, C.E.3    Cole, S.P.C.4    Deeley, R.G.5
  • 14
    • 0029024314 scopus 로고
    • Cloning of the β cell high affinity sulfonylurea receptor: A regulator of insulin secretion
    • 14. Aguilar-Bryan L, Nichols CG, Wechsler SW, et al. Cloning of the β cell high affinity sulfonylurea receptor: a regulator of insulin secretion. Science. 1995;268:423-426.
    • (1995) Science , vol.268 , pp. 423-426
    • Aguilar-Bryan, L.1    Nichols, C.G.2    Wechsler, S.W.3
  • 16
    • 0029021696 scopus 로고
    • Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy
    • 16. Thomas PM, Cote GJ, Wohlik N, et al. Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. Science. 1995;268:426-429.
    • (1995) Science , vol.268 , pp. 426-429
    • Thomas, P.M.1    Cote, G.J.2    Wohlik, N.3
  • 17
    • 0026593016 scopus 로고
    • Assembly and function of the two ABC transporters encoded in the human major histocompatibility complex
    • 17. Kelly A, Powis SH, Kerr LA, et al. Assembly and function of the two ABC transporters encoded in the human major histocompatibility complex. Nature. 1992;355:641-644.
    • (1992) Nature , vol.355 , pp. 641-644
    • Kelly, A.1    Powis, S.H.2    Kerr, L.A.3
  • 18
    • 0029591999 scopus 로고
    • Association of HLA class I antigen deficiency related to a TAP2 gene mutation with familial bronchiectasis
    • 18. Donato L, de la Salle D, Hanau D, et al. Association of HLA class I antigen deficiency related to a TAP2 gene mutation with familial bronchiectasis. J Pediatr. 1995;127:895-900.
    • (1995) J Pediatr. , vol.127 , pp. 895-900
    • Donato, L.1    De La Salle, D.2    Hanau, D.3
  • 19
    • 0027532282 scopus 로고
    • Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters
    • 19. Mosser J, Douar A-M, Sarde C-O, et al. Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters. Nature. 1993;361:726-730.
    • (1993) Nature , vol.361 , pp. 726-730
    • Mosser, J.1    Douar, A.-M.2    Sarde, C.-O.3
  • 20
    • 0027978453 scopus 로고
    • Identification of mutations in the putative ATP-binding domain of the adrenoleukodystrophy gene
    • 20. Fanen P, Guidoux S, Sarde C-O, et al. Identification of mutations in the putative ATP-binding domain of the adrenoleukodystrophy gene. J Clin Invest. 1994;94:516-520.
    • (1994) J Clin Invest. , vol.94 , pp. 516-520
    • Fanen, P.1    Guidoux, S.2    Sarde, C.-O.3
  • 21
    • 0029894981 scopus 로고    scopus 로고
    • Molecular cloning and expression of a cyclic AMP-activated chloride conductance regulator: A novel ATP-binding cassette transporter
    • 21. van Kuijck MA, van Aubel RAMH, Busch AE, et al. Molecular cloning and expression of a cyclic AMP-activated chloride conductance regulator: a novel ATP-binding cassette transporter. Proc Natl Acad Sci USA. 1996;93:5401-5406.
    • (1996) Proc Natl Acad Sci USA , vol.93 , pp. 5401-5406
    • Van Kuijck, M.A.1    Van Aubel, R.A.M.H.2    Busch, A.E.3
  • 22
    • 0026763138 scopus 로고
    • Cystic fibrosis transmembrane conductance regulator: A chloride channel with novel regulation
    • 22. Welsh MJ, Anderson MP, Rich DP, et al. Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation. Neuron. 1992;8:821-829.
    • (1992) Neuron , vol.8 , pp. 821-829
    • Welsh, M.J.1    Anderson, M.P.2    Rich, D.P.3
  • 24
    • 0031046796 scopus 로고    scopus 로고
    • Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A
    • 24. Jia Y, Mathews CJ, Hanrahan JW. Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A. J Biol Chem. 1997;272:4978-4984.
    • (1997) J Biol Chem. , vol.272 , pp. 4978-4984
    • Jia, Y.1    Mathews, C.J.2    Hanrahan, J.W.3
  • 25
    • 0010444718 scopus 로고    scopus 로고
    • CFTR is a "conductance regulator" as well as a chloride channel
    • in press
    • 25. Schwiebert EM, Benos DJ, Egan ME, et al. CFTR is a "conductance regulator" as well as a chloride channel. Physiol Rev. 1998; in press.
    • (1998) Physiol Rev.
    • Schwiebert, E.M.1    Benos, D.J.2    Egan, M.E.3
  • 26
    • 0028980536 scopus 로고
    • CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP
    • 26. Schwiebert EM, Egan ME, Hwang T-H, et al. CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell. 1995;81:1063-1073.
    • (1995) Cell , vol.81 , pp. 1063-1073
    • Schwiebert, E.M.1    Egan, M.E.2    Hwang, T.-H.3
  • 27
    • 0023213846 scopus 로고
    • Permeability properties of cell membranes and tight junctions of normal and cystic fibrosis sweat ducts
    • 27. Bijman J, Quinton PM. Permeability properties of cell membranes and tight junctions of normal and cystic fibrosis sweat ducts. Pflugers Arch. 1987;408:505-510.
    • (1987) Pflugers Arch. , vol.408 , pp. 505-510
    • Bijman, J.1    Quinton, P.M.2
  • 28
    • 0000810353 scopus 로고
    • Chloride channels along the nephron
    • Guggino WB, ed. San Diego: Academic Press
    • 28. Schwiebert EM, Lopes AG, Guggino WB. Chloride channels along the nephron. In: Guggino WB, ed. Chloride Channels. San Diego: Academic Press; 1994:265-315.
    • (1994) Chloride Channels , pp. 265-315
    • Schwiebert, E.M.1    Lopes, A.G.2    Guggino, W.B.3
  • 29
    • 0010484689 scopus 로고    scopus 로고
    • Abnormal chloride and sodium channel function in cystic fibrosis airway epithelia
    • Crystal RG, Barnes PJ, West JB, Weibel ER, eds. New York: Lippincott-Raven
    • 29. Schwiebert EM, Guggino WB. Abnormal chloride and sodium channel function in cystic fibrosis airway epithelia. In: Crystal RG, Barnes PJ, West JB, Weibel ER, eds. The Lung: Scientific Foundations. New York: Lippincott-Raven; 1997:2555-2572.
    • (1997) The Lung: Scientific Foundations , pp. 2555-2572
    • Schwiebert, E.M.1    Guggino, W.B.2
  • 31
    • 85047691737 scopus 로고
    • CFTR, a channel with the structure of a transporter
    • 31. Riordan JR, Chang X-B. CFTR, a channel with the structure of a transporter. Biochim Biophys Acta. 1992;1101:221-222.
    • (1992) Biochim Biophys Acta , vol.1101 , pp. 221-222
    • Riordan, J.R.1    Chang, X.-B.2
  • 32
    • 0026054703 scopus 로고
    • - channel activity in Xenopus oocytes expressing the cystic fibrosis gene
    • - channel activity in Xenopus oocytes expressing the cystic fibrosis gene. J Biol Chem. 1991;266:19142-19145.
    • (1991) J Biol Chem. , vol.266 , pp. 19142-19145
    • Bear, C.E.1    Duguay, F.2    Naismith, A.L.3
  • 33
    • 0025094612 scopus 로고
    • Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer
    • 33. Drumm ML, Pope HA, Cliff WH, et al. Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer. Cell. 1990;62:1227-1233.
    • (1990) Cell , vol.62 , pp. 1227-1233
    • Drumm, M.L.1    Pope, H.A.2    Cliff, W.H.3
  • 34
    • 0025863209 scopus 로고
    • Generation of cAMP-activated chloride currents by expression of CFTR
    • 34. Anderson MP, Rich DP, Gregory RJ, et al. Generation of cAMP-activated chloride currents by expression of CFTR. Science. 1991; 251:679-682.
    • (1991) Science , vol.251 , pp. 679-682
    • Anderson, M.P.1    Rich, D.P.2    Gregory, R.J.3
  • 35
    • 0026699623 scopus 로고
    • Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers
    • 35. Tilly BC, Winter MC, Ostedgaard LS, et al. Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers. J Biol Chem. 1992;267:9470-9473.
    • (1992) J Biol Chem. , vol.267 , pp. 9470-9473
    • Tilly, B.C.1    Winter, M.C.2    Ostedgaard, L.S.3
  • 36
    • 0026532895 scopus 로고
    • Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
    • 36. Bear CE, Li C, Kartner N, et al. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell. 1992;68:809-818.
    • (1992) Cell , vol.68 , pp. 809-818
    • Bear, C.E.1    Li, C.2    Kartner, N.3
  • 37
    • 0026077676 scopus 로고
    • Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel
    • 37. Berger HA, Anderson MP, Gregory RJ, et al. Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel. J Clin Invest. 1991;88:1422-1431.
    • (1991) J Clin Invest. , vol.88 , pp. 1422-1431
    • Berger, H.A.1    Anderson, M.P.2    Gregory, R.J.3
  • 38
    • 0025931429 scopus 로고
    • Nucleoside triphosphates are required to open the CFTR chloride channel
    • 38. Anderson MP, Berger HA, Rich DP, et al. Nucleoside triphosphates are required to open the CFTR chloride channel. Cell. 1991; 67:775-784.
    • (1991) Cell , vol.67 , pp. 775-784
    • Anderson, M.P.1    Berger, H.A.2    Rich, D.P.3
  • 39
    • 0023931773 scopus 로고
    • Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium
    • 39. Li M, McCann JD, Liedtke CM, et al. Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium. Nature. 1988;331:358-360.
    • (1988) Nature , vol.331 , pp. 358-360
    • Li, M.1    McCann, J.D.2    Liedtke, C.M.3
  • 40
    • 0023546389 scopus 로고
    • Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells
    • 40. Schoumacher RA, Shoemaker RL, Halm DR, et al. Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells. Nature. 1987;330:752-754.
    • (1987) Nature , vol.330 , pp. 752-754
    • Schoumacher, R.A.1    Shoemaker, R.L.2    Halm, D.R.3
  • 41
    • 0024353683 scopus 로고
    • Chloride channels in CF: Lack of activation by protein kinase C and cAMP-dependent protein kinase
    • 41. Hwang T-C, Lu L, Zeitlin, PL, et al. Chloride channels in CF: lack of activation by protein kinase C and cAMP-dependent protein kinase. Science. 1989;244:1351-1353.
    • (1989) Science , vol.244 , pp. 1351-1353
    • Hwang, T.-C.1    Lu, L.2    Zeitlin, P.L.3
  • 42
    • 0027537498 scopus 로고
    • - channels in undifferentiated human colonic cells (HT-29). I. Single-channel properties
    • - channels in undifferentiated human colonic cells (HT-29). I. Single-channel properties. Am J Physiol (Cell Physiol). 1993;264:C968-C976.
    • (1993) Am J Physiol (Cell Physiol) , vol.264
    • Morris, A.P.1    Frizzell, R.A.2
  • 43
    • 0028026796 scopus 로고
    • Molecular physiology of chloride channels
    • 43. Jentsch TJ. Molecular physiology of chloride channels. Curr Opin Cell Biol. 1994;6:600-606.
    • (1994) Curr Opin Cell Biol. , vol.6 , pp. 600-606
    • Jentsch, T.J.1
  • 44
    • 0027996879 scopus 로고
    • Molecular physiology of voltage-gated chloride channels
    • 44. Pusch M, Jentsch TJ. Molecular physiology of voltage-gated chloride channels. Physiol Rev. 1994;74:813-827.
    • (1994) Physiol Rev. , vol.74 , pp. 813-827
    • Pusch, M.1    Jentsch, T.J.2
  • 45
    • 0343812098 scopus 로고    scopus 로고
    • Chloride channels: An emerging molecular picture
    • 45. Jentsch TJ, Gunther W. Chloride channels: an emerging molecular picture. BioEssays. 1997;19:117-126.
    • (1997) BioEssays , vol.19 , pp. 117-126
    • Jentsch, T.J.1    Gunther, W.2
  • 46
    • 0026687233 scopus 로고
    • - channels by protein kinase A corrected by insertion of CFTR
    • - channels by protein kinase A corrected by insertion of CFTR. Nature. 1992;358:581-584.
    • (1992) Nature , vol.358 , pp. 581-584
    • Egan, M.E.1    Flotte, T.2    Afione, S.3
  • 47
    • 0027191065 scopus 로고
    • CFTR and outwardly rectifying chloride channels are distinct proteins with a regulatory relationship
    • 47. Gabriel SE, Clarke LL, Boucher RC, Stutts MJ. CFTR and outwardly rectifying chloride channels are distinct proteins with a regulatory relationship. Nature. 1993;363:263-268.
    • (1993) Nature , vol.363 , pp. 263-268
    • Gabriel, S.E.1    Clarke, L.L.2    Boucher, R.C.3    Stutts, M.J.4
  • 48
    • 0028899752 scopus 로고
    • Cystic fibrosis transmembrane conductance regulator is required for protein kinase activation of an outwardly rectified anion channel purified from bovine tracheal epithelia
    • 48. Jovov B, Ismailov II, Benos DJ. Cystic fibrosis transmembrane conductance regulator is required for protein kinase activation of an outwardly rectified anion channel purified from bovine tracheal epithelia. J Biol Chem. 1995;270:1521-1528.
    • (1995) J Biol Chem. , vol.270 , pp. 1521-1528
    • Jovov, B.1    Ismailov, I.I.2    Benos, D.J.3
  • 49
    • 0029785898 scopus 로고    scopus 로고
    • Apical and basolateral ATP stimulate tracheal epithelial chloride secretion via multiple purinergic receptors and signaling pathways
    • 49. Hwang TH, Schwiebert EM, Guggino WB. Apical and basolateral ATP stimulate tracheal epithelial chloride secretion via multiple purinergic receptors and signaling pathways. Am J Physiol (Cell Physiol). 1996;270:C1611-C1623.
    • (1996) Am J Physiol (Cell Physiol) , vol.270
    • Hwang, T.H.1    Schwiebert, E.M.2    Guggino, W.B.3
  • 50
    • 0028810639 scopus 로고
    • Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels
    • 50. Jovov B, Ismailov II, Berdiev BK, et al. Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels. J Biol Chem. 1995;270:29194-29200.
    • (1995) J Biol Chem. , vol.270 , pp. 29194-29200
    • Jovov, B.1    Ismailov, I.I.2    Berdiev, B.K.3
  • 51
    • 0026640380 scopus 로고
    • Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis
    • 51. Clarke LL, Grubb BR, Gabriel SE, et al. Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science. 1992;257:1125-1128.
    • (1992) Science , vol.257 , pp. 1125-1128
    • Clarke, L.L.1    Grubb, B.R.2    Gabriel, S.E.3
  • 54
    • 0026591559 scopus 로고
    • Extracellular ATP and UTP induce chloride secretion in nasal epithelia of cystic fibrosis patients and normal subjects in vivo
    • 54. Knowles MR, Clarke LL, Boucher RC. Extracellular ATP and UTP induce chloride secretion in nasal epithelia of cystic fibrosis patients and normal subjects in vivo. Chest. 1992;101:60-63.
    • (1992) Chest , vol.101 , pp. 60-63
    • Knowles, M.R.1    Clarke, L.L.2    Boucher, R.C.3
  • 55
    • 0029887172 scopus 로고    scopus 로고
    • Effect of uridine 5′-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis
    • 55. Bennett WD, Olivier KN, Zeman KL, et al. Effect of uridine 5′-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis. Am J Respir Crit Care Med. 1996;153:1796-1801.
    • (1996) Am J Respir Crit Care Med. , vol.153 , pp. 1796-1801
    • Bennett, W.D.1    Olivier, K.N.2    Zeman, K.L.3
  • 56
    • 0022973473 scopus 로고
    • + transport in cystic fibrosis epithelia. Abnormal basal rate and response to adenylate cyclase activation
    • + transport in cystic fibrosis epithelia. Abnormal basal rate and response to adenylate cyclase activation. J Clin Invest. 1986;78:1245-2152.
    • (1986) J Clin Invest. , vol.78 , pp. 1245-2152
    • Boucher, R.C.1    Stutts, M.J.2    Knowles, M.R.3
  • 57
    • 0020610435 scopus 로고
    • Abnormal ion permeation through cystic fibrosis respiratory epithelium
    • 57. Knowles MR, Stutts MJ, Spock A, et al. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science. 1983;221: 1067-1070.
    • (1983) Science , vol.221 , pp. 1067-1070
    • Knowles, M.R.1    Stutts, M.J.2    Spock, A.3
  • 58
    • 0027483065 scopus 로고
    • Epithelial sodium channel related to proteins involved in neurodegradation
    • 58. Canessa CM, Horisberger J-D, Rossier BC. Epithelial sodium channel related to proteins involved in neurodegradation. Nature. 1993;361:467-470.
    • (1993) Nature , vol.361 , pp. 467-470
    • Canessa, C.M.1    Horisberger, J.-D.2    Rossier, B.C.3
  • 59
    • 0027958441 scopus 로고
    • + channel is made of three homologous subunits
    • + channel is made of three homologous subunits. Nature. 1994; 367:463-467.
    • (1994) Nature , vol.367 , pp. 463-467
    • Canessa, C.M.1    Schild, L.2    Buell, G.3
  • 62
    • 0029159758 scopus 로고
    • Increased expression and activity of sodium channels in alveolar type II cells of hyperoxic rats
    • 62. Yue G, Russell WJ, Benos DJ, et al. Increased expression and activity of sodium channels in alveolar type II cells of hyperoxic rats. Proc Natl Acad Sci USA 1995;92:8418-8422.
    • (1995) Proc Natl Acad Sci USA , vol.92 , pp. 8418-8422
    • Yue, G.1    Russell, W.J.2    Benos, D.J.3
  • 63
    • 0028276637 scopus 로고
    • Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: A patch-clamp study
    • 63. Chinet TC, Fullton JM, Yankaskas JR, et al. Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: a patch-clamp study. Am J Physiol (Cell Physiol). 1994;266:C1061-C1068.
    • (1994) Am J Physiol (Cell Physiol) , vol.266
    • Chinet, T.C.1    Fullton, J.M.2    Yankaskas, J.R.3
  • 64
    • 0024786312 scopus 로고
    • Cystic fibrosis affects chloride and sodium channels in human airway epithelia
    • 64. Duszyk M, French AS, Man SFP. Cystic fibrosis affects chloride and sodium channels in human airway epithelia. Can J Physiol Pharmacol. 1989;67:1362-1365.
    • (1989) Can J Physiol Pharmacol. , vol.67 , pp. 1362-1365
    • Duszyk, M.1    French, A.S.2    Man, S.F.P.3
  • 67
    • 0026520186 scopus 로고
    • + channels: Function and diversity
    • + channels: function and diversity. Annu Rev Physiol. 1992;54:51-66.
    • (1992) Annu Rev Physiol. , vol.54 , pp. 51-66
    • Palmer, L.G.1
  • 68
    • 0030898750 scopus 로고    scopus 로고
    • Cyclic nucleotide-gated chloride and sodium currents in rat tracheal airway epithelium
    • 68. Schwiebert EM, Potter ED, Hwang T-H, et al. Cyclic nucleotide-gated chloride and sodium currents in rat tracheal airway epithelium. Am J Physiol (Cell Physiol). 1997;272:C911-C922.
    • (1997) Am J Physiol (Cell Physiol) , vol.272
    • Schwiebert, E.M.1    Potter, E.D.2    Hwang, T.-H.3
  • 69
    • 0028912888 scopus 로고
    • Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelia cells
    • 69. Johnson LG, Boyles SE, Wilson JM, Boucher RC. Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelia cells. J Clin Invest. 1995;95:1377-1382.
    • (1995) J Clin Invest. , vol.95 , pp. 1377-1382
    • Johnson, L.G.1    Boyles, S.E.2    Wilson, J.M.3    Boucher, R.C.4
  • 70
    • 0028982894 scopus 로고
    • CFTR as a cAMP-dependent regulator of sodium channels
    • 70. Stutts MJ, Canessa CM, Olsen JC, et al. CFTR as a cAMP-dependent regulator of sodium channels. Science. 1995;269:847-850.
    • (1995) Science , vol.269 , pp. 847-850
    • Stutts, M.J.1    Canessa, C.M.2    Olsen, J.C.3
  • 71
    • 0030003079 scopus 로고    scopus 로고
    • Regulation of epithelial sodium channels by short actin filaments
    • 71. Berdiev BK, Prat AG, Cantiello HF, et al. Regulation of epithelial sodium channels by short actin filaments. J Biol Chem. 1996;271: 17704-17710.
    • (1996) J Biol Chem. , vol.271 , pp. 17704-17710
    • Berdiev, B.K.1    Prat, A.G.2    Cantiello, H.F.3
  • 72
    • 0029912194 scopus 로고    scopus 로고
    • Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator
    • 72. Ismailov II, Awayda MS, Jovov B, et al. Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator. J Biol Chem. 1996;271:4725-4732.
    • (1996) J Biol Chem. , vol.271 , pp. 4725-4732
    • Ismailov, I.I.1    Awayda, M.S.2    Jovov, B.3
  • 74
    • 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. 1997; 400:341-344.
    • (1997) FEBS Lett. , vol.400 , pp. 341-344
    • Kunzelmann, K.1    Kiser, G.L.2    Schreiber, R.3    Riordan, J.R.4
  • 75
    • 0019985202 scopus 로고
    • Measurements of transepithelial electric potential differences in the trachea and bronchi of human subjects in vivo
    • 75. Knowles MR, Buntin WH, Bromberg PA, et al. Measurements of transepithelial electric potential differences in the trachea and bronchi of human subjects in vivo. Am Rev Respir Dis. 1982;126: 108-112.
    • (1982) Am Rev Respir Dis. , vol.126 , pp. 108-112
    • Knowles, M.R.1    Buntin, W.H.2    Bromberg, P.A.3
  • 76
    • 0025784535 scopus 로고
    • Defective acidification of intracellular organelles in cystic fibrosis
    • 76. Barasch J, Kiss B, Prince A, et al. Defective acidification of intracellular organelles in cystic fibrosis. Nature. 1991;352:70-73.
    • (1991) Nature , vol.352 , pp. 70-73
    • Barasch, J.1    Kiss, B.2    Prince, A.3
  • 77
    • 0027763241 scopus 로고
    • Defective acidification of the biosynthetic pathway in cystic fibrosis
    • 77. Barasch J, Al-Awqati Q. Defective acidification of the biosynthetic pathway in cystic fibrosis. J Cell Sci. 1993;17:229-233.
    • (1993) J Cell Sci. , vol.17 , pp. 229-233
    • Barasch, J.1    Al-Awqati, Q.2
  • 78
    • 0026581842 scopus 로고
    • Regulation of plasma membrane recycling by CFTR
    • 78. Bradbury NA, Jilling T, Berta G, et al. Regulation of plasma membrane recycling by CFTR. Science. 1992;256:530-532.
    • (1992) Science , vol.256 , pp. 530-532
    • Bradbury, N.A.1    Jilling, T.2    Berta, G.3
  • 80
    • 0028140935 scopus 로고
    • Heterotrimeric g proteins, vesicle trafficking, and CFTR chloride channels
    • 80. Schwiebert EM, Gesek F, Ercolani L, et al. Heterotrimeric G proteins, vesicle trafficking, and CFTR chloride channels. Am J Physiol (Cell Physiol). 1994;267:C272-C281.
    • (1994) Am J Physiol (Cell Physiol) , vol.267
    • Schwiebert, E.M.1    Gesek, F.2    Ercolani, L.3
  • 81
    • 0028920643 scopus 로고
    • Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface
    • 81. Imundo L, Barasch J, Prince A, Al-Awqati Q. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci USA. 1995;92:3019-3025.
    • (1995) Proc Natl Acad Sci USA , vol.92 , pp. 3019-3025
    • Imundo, L.1    Barasch, J.2    Prince, A.3    Al-Awqati, Q.4
  • 82
    • 0027390125 scopus 로고
    • Effects of the deltaF508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR
    • 82. Thomas PJ, Pedersen PL. Effects of the deltaF508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR. J Bioenerg Biomembr. 1993;25:11-19.
    • (1993) J Bioenerg Biomembr. , vol.25 , pp. 11-19
    • Thomas, P.J.1    Pedersen, P.L.2
  • 83
    • 0028856292 scopus 로고
    • Defective protein folding as a basis of human disease
    • 83. Thomas PJ, Qu B-H, Pedersen PL. Defective protein folding as a basis of human disease. Trends Biochem Sci. 1995;20:456-459.
    • (1995) Trends Biochem Sci. , vol.20 , pp. 456-459
    • Thomas, P.J.1    Qu, B.-H.2    Pedersen, P.L.3
  • 84
    • 0025310336 scopus 로고
    • A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator protein
    • 84. Cutting GR, Kasch LM, Rosenstein BJ, et al. A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator protein. Nature. 1990;346:366-369.
    • (1990) Nature , vol.346 , pp. 366-369
    • Cutting, G.R.1    Kasch, L.M.2    Rosenstein, B.J.3
  • 85
    • 0026732670 scopus 로고
    • Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis
    • 85. McIntosh I, Cutting GR. Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis. FASEB J 1992;6:2775-2782.
    • (1992) FASEB J , vol.6 , pp. 2775-2782
    • McIntosh, I.1    Cutting, G.R.2
  • 86
    • 0026937999 scopus 로고
    • CFTR nonsense mutations G542X and W1282X associated with severe reduction in CFTR mRNA in nasal epithelial cells
    • 86. Hamosh A, Rosenstein BJ, Cutting GR. CFTR nonsense mutations G542X and W1282X associated with severe reduction in CFTR mRNA in nasal epithelial cells. Hum Mol Genet. 1992;1:542-544.
    • (1992) Hum Mol Genet. , vol.1 , pp. 542-544
    • Hamosh, A.1    Rosenstein, B.J.2    Cutting, G.R.3
  • 87
    • 0026322140 scopus 로고
    • Severe deficiency in cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis
    • 87. Hamosh A, Trapnell BC, Zeitlin PL, et al. Severe deficiency in cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis. J Clin Invest. 1991;88:1880-1885.
    • (1991) J Clin Invest. , vol.88 , pp. 1880-1885
    • Hamosh, A.1    Trapnell, B.C.2    Zeitlin, P.L.3
  • 88
    • 0029118117 scopus 로고
    • Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents
    • 88. Fulmer SB, Schwiebert EM, Morales MM, et al. Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents. Proc Natl Acad Sci USA. 1995;92:6832-6836.
    • (1995) Proc Natl Acad Sci USA , vol.92 , pp. 6832-6836
    • Fulmer, S.B.1    Schwiebert, E.M.2    Morales, M.M.3
  • 89
    • 0027364318 scopus 로고
    • Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator
    • 89. Smit LS, Wilkinson DJ, Mansoura MK, et al. Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci USA. 1993;90:9963-9967.
    • (1993) Proc Natl Acad Sci USA , vol.90 , pp. 9963-9967
    • Smit, L.S.1    Wilkinson, D.J.2    Mansoura, M.K.3
  • 90
    • 0026337305 scopus 로고
    • Chloride channels expressed by delF508 and other mutant CFTRs in Xenopus oocytes
    • 90. Drumm ML, Wilkinson DJ, Smit L, et al. Chloride channels expressed by delF508 and other mutant CFTRs in Xenopus oocytes. Science. 1991;254:1797-1799.
    • (1991) Science , vol.254 , pp. 1797-1799
    • Drumm, M.L.1    Wilkinson, D.J.2    Smit, L.3
  • 91
    • 0025967994 scopus 로고
    • Association of less common cystic fibrosis mutations with a mild phenotype
    • 91. Curtis A, Nelson R, Porteous M, et al. Association of less common cystic fibrosis mutations with a mild phenotype. J Med Genet. 1991;28:34-37.
    • (1991) J Med Genet. , vol.28 , pp. 34-37
    • Curtis, A.1    Nelson, R.2    Porteous, M.3
  • 92
    • 0029766205 scopus 로고    scopus 로고
    • Heterogeneity of phenotype in two cystic fibrosis patients homozygous for the CFTR exon 11 mutation G551D
    • 92. Parad RB. Heterogeneity of phenotype in two cystic fibrosis patients homozygous for the CFTR exon 11 mutation G551D. J Med Genet. 1996;33:711-713.
    • (1996) J Med Genet. , vol.33 , pp. 711-713
    • Parad, R.B.1
  • 93
    • 9044236531 scopus 로고    scopus 로고
    • Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations
    • 93. Delaney SJ, Alton EW, Smith SN, et al. Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations. J Med Genet. 1996;15:955-963.
    • (1996) J Med Genet. , vol.15 , pp. 955-963
    • Delaney, S.J.1    Alton, E.W.2    Smith, S.N.3
  • 94
    • 0028366412 scopus 로고
    • Transmembrane mutations alter the channel characteristics of the cystic fibrosis transmembrane conductance regulator expressed in Xenopus oocytes
    • 94. Piazza-Carroll T, McIntosh I, Egan ME, et al. Transmembrane mutations alter the channel characteristics of the cystic fibrosis transmembrane conductance regulator expressed in Xenopus oocytes. Cell Physiol Biochem. 1994;4:10-18.
    • (1994) Cell Physiol Biochem. , vol.4 , pp. 10-18
    • Piazza-Carroll, T.1    McIntosh, I.2    Egan, M.E.3
  • 95
    • 0028069337 scopus 로고
    • The genetic basis of congenital bilateral absence of the vas deferens and cystic fibrosis
    • 95. Oates RD, Amos JA. The genetic basis of congenital bilateral absence of the vas deferens and cystic fibrosis. J Androl. 1994;15:1-8.
    • (1994) J Androl. , vol.15 , pp. 1-8
    • Oates, R.D.1    Amos, J.A.2
  • 96
    • 16044362187 scopus 로고    scopus 로고
    • Diversity of reproductive tract abnormalities in men with cystic fibrosis
    • 96. Wilschanski M, Corey M, Durie P, et al. Diversity of reproductive tract abnormalities in men with cystic fibrosis. JAMA. 1996;276: 607-608.
    • (1996) JAMA , vol.276 , pp. 607-608
    • Wilschanski, M.1    Corey, M.2    Durie, P.3
  • 98
    • 0027423190 scopus 로고
    • Multi-ion pore behaviour in the CFTR chloride channel
    • 98. Tabcharani JA, Rommens JM, Hou Y-X, et al. Multi-ion pore behaviour in the CFTR chloride channel. Nature. 1993;366:79-82.
    • (1993) Nature , vol.366 , pp. 79-82
    • Tabcharani, J.A.1    Rommens, J.M.2    Hou, Y.-X.3
  • 99
    • 0010446791 scopus 로고
    • A disease-related mutation in the fifth putative transmembrane segments alters the conduction and gating properties of CFTR
    • Abstract
    • 99. Mansoura MK, Strong TV, Collins FS, Dawson DC. A disease-related mutation in the fifth putative transmembrane segments alters the conduction and gating properties of CFTR. J Gen Physiol. 1994;104:36a. Abstract.
    • (1994) J Gen Physiol. , vol.104
    • Mansoura, M.K.1    Strong, T.V.2    Collins, F.S.3    Dawson, D.C.4
  • 100
    • 17544374096 scopus 로고    scopus 로고
    • Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity
    • 100. Seibert FS, Linsdell P, Loo TW, et al. Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity. JBiol Chem. 1996;271:15139-15145.
    • (1996) J Biol Chem. , vol.271 , pp. 15139-15145
    • Seibert, F.S.1    Linsdell, P.2    Loo, T.W.3
  • 101
    • 0025987020 scopus 로고
    • Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel
    • 101. Cheng SH, Rich DP, Marshall J, et al. Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel. Cell. 1991;66:1027-1036.
    • (1991) Cell , vol.66 , pp. 1027-1036
    • Cheng, S.H.1    Rich, D.P.2    Marshall, J.3
  • 102
    • 0025744023 scopus 로고
    • Effect of deleting the R domain on CFTR-generated chloride channels
    • 102. Rich DP, Gregory RJ, Anderson MP, et al. Effect of deleting the R domain on CFTR-generated chloride channels. Science. 1991;253: 205-207.
    • (1991) Science , vol.253 , pp. 205-207
    • Rich, D.P.1    Gregory, R.J.2    Anderson, M.P.3
  • 103
    • 0027311276 scopus 로고
    • Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites
    • 103. Chang X-B, Tabcharani JA, Hou Y-X, et al. Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. J Biol Chem. 1993; 268:11304-11311.
    • (1993) J Biol Chem. , vol.268 , pp. 11304-11311
    • Chang, X.-B.1    Tabcharani, J.A.2    Hou, Y.-X.3
  • 104
    • 0027278897 scopus 로고
    • Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis
    • 104. Ramsey BW, Astley SJ, Aitken ML, et al. Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis. Am Rev Respir Dis. 1993;148:145-151.
    • (1993) Am Rev Respir Dis. , vol.148 , pp. 145-151
    • Ramsey, B.W.1    Astley, S.J.2    Aitken, M.L.3
  • 105
    • 0028212809 scopus 로고
    • Reduction of viscosity of cystic fibrosis sputum in vitro by gelsolin
    • 105. Vasconcellos CA, Allen PG, Wohl ME, et al. Reduction of viscosity of cystic fibrosis sputum in vitro by gelsolin. Science. 1994;263: 969-971.
    • (1994) Science , vol.263 , pp. 969-971
    • Vasconcellos, C.A.1    Allen, P.G.2    Wohl, M.E.3
  • 106
    • 0028914667 scopus 로고
    • Effect of high dose ibuprofen in patients with cystic fibrosis
    • 106. Konstan MW, Byard PJ, Hoppel CL, Davis PB. Effect of high dose ibuprofen in patients with cystic fibrosis. NEJM. 1995;332:848-854.
    • (1995) NEJM , vol.332 , pp. 848-854
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3    Davis, P.B.4
  • 107
    • 0029870085 scopus 로고    scopus 로고
    • Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
    • 107. Smith JJ, Travis SM, Greenberg EP, Welsh MJ. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell. 1996;85:229-236.
    • (1996) Cell , vol.85 , pp. 229-236
    • Smith, J.J.1    Travis, S.M.2    Greenberg, E.P.3    Welsh, M.J.4
  • 108
    • 0030914575 scopus 로고    scopus 로고
    • A sensitive defense: Salt and cystic fibrosis
    • 108. Wine JJ. A sensitive defense: salt and cystic fibrosis. Nature Med. 1997;3:494-495.
    • (1997) Nature Med. , vol.3 , pp. 494-495
    • Wine, J.J.1
  • 109
    • 0031093274 scopus 로고    scopus 로고
    • Human airway epithelia express a β-defensin
    • 109. McCray PB, Bentley L. Human airway epithelia express a β-defensin. Am J Resp Cell Mol Biol. 1997;16:343-349.
    • (1997) Am J Resp Cell Mol Biol. , vol.16 , pp. 343-349
    • McCray, P.B.1    Bentley, L.2
  • 110
    • 0030949875 scopus 로고    scopus 로고
    • Human β-defensin-1 is a salt-sensitive antibiotic in lung that is inactivated in cystic fibrosis
    • 110. Goldman MJ, Anderson GM, Stolzenberg ED, et al. Human β-defensin-1 is a salt-sensitive antibiotic in lung that is inactivated in cystic fibrosis. Cell. 1997;88:553-560.
    • (1997) Cell , vol.88 , pp. 553-560
    • Goldman, M.J.1    Anderson, G.M.2    Stolzenberg, E.D.3
  • 111
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature sensitive
    • 111. Denning GM, Anderson MP, Amara JF, et al. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature sensitive. Nature. 1992;358:761-764.
    • (1992) Nature , vol.358 , pp. 761-764
    • Denning, G.M.1    Anderson, M.P.2    Amara, J.F.3
  • 112
    • 0028840915 scopus 로고
    • Degradation of CFTR by the ubiquitin-proteasome pathway
    • 112. Ward CL, Omura S, Kopito RR. Degradation of CFTR by the ubiquitin-proteasome pathway. Cell. 1995;83:121-127.
    • (1995) Cell , vol.83 , pp. 121-127
    • Ward, C.L.1    Omura, S.2    Kopito, R.R.3
  • 113
    • 0028858161 scopus 로고
    • Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
    • 113. Jensen TJ, Loo MA, Pind S, et al. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell. 1995;83:129-135.
    • (1995) Cell , vol.83 , pp. 129-135
    • Jensen, T.J.1    Loo, M.A.2    Pind, S.3
  • 114
    • 0010442552 scopus 로고    scopus 로고
    • Clinical trials of 4-phenylbutyrate for correction of sweat duct abnormalities in delF508 homozygous cystic fibrosis patients
    • 114. Rubenstein RC, Brusilow SW, Hamosh A, Zeitlin PL. Clinical trials of 4-phenylbutyrate for correction of sweat duct abnormalities in delF508 homozygous cystic fibrosis patients. Ped Pulmonol 1996;13:259.
    • (1996) Ped Pulmonol , vol.13 , pp. 259
    • Rubenstein, R.C.1    Brusilow, S.W.2    Hamosh, A.3    Zeitlin, P.L.4
  • 115
    • 0029994529 scopus 로고    scopus 로고
    • Aminoglycoside antibiotics restore CFTR function by overcoming premature stop codon mutations
    • 115. Howard M, Frizzell RA, Bedwell DM. Aminoglycoside antibiotics restore CFTR function by overcoming premature stop codon mutations. Nature Med. 1996;2:467-469.
    • (1996) Nature Med. , vol.2 , pp. 467-469
    • Howard, M.1    Frizzell, R.A.2    Bedwell, D.M.3
  • 116
    • 0028812220 scopus 로고
    • Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis
    • 116. Knowles MR. Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis. Chest. 1995;107:71S-76S.
    • (1995) Chest , vol.107
    • Knowles, M.R.1
  • 117
    • 0030901674 scopus 로고    scopus 로고
    • Pharmacokinetics of amiloride after inhalation and oral administration in adolescents and adults with cystic fibrosis
    • 117. Jones KM, Liao E, Hohneker KW, et al. Pharmacokinetics of amiloride after inhalation and oral administration in adolescents and adults with cystic fibrosis. Pharmacotherapy. 1997;17:263-270.
    • (1997) Pharmacotherapy , vol.17 , pp. 263-270
    • Jones, K.M.1    Liao, E.2    Hohneker, K.W.3
  • 118
    • 0025236867 scopus 로고
    • A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
    • 118. Knowles MR, Church NL, Waltner WE, et al. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. NEJM. 1990;322:1189-1194.
    • (1990) NEJM , vol.322 , pp. 1189-1194
    • Knowles, M.R.1    Church, N.L.2    Waltner, W.E.3
  • 119
    • 0028825747 scopus 로고
    • Gene therapy for cystic fibrosis: Challenges and future directions
    • 119. Wilson JM. Gene therapy for cystic fibrosis: challenges and future directions. J Clin Invest. 1995;96:2547-2554.
    • (1995) J Clin Invest. , vol.96 , pp. 2547-2554
    • Wilson, J.M.1
  • 120
    • 0040982343 scopus 로고    scopus 로고
    • A complete catalogue of Saccharomyces cerevisiae ABC proteins and their relevance to human health and disease
    • Ambudkar SV, Gottesman MM, eds. San Diego, California: Academic Press
    • 120. Taglicht D, Michaelis S. A complete catalogue of Saccharomyces cerevisiae ABC proteins and their relevance to human health and disease. In: Ambudkar SV, Gottesman MM, eds. ABC Transporters: Biochemical, Cellular, and Molecular Aspects. Methods in Enzymology, Volume 292, San Diego, California: Academic Press, 1998.
    • (1998) ABC Transporters: Biochemical, Cellular, and Molecular Aspects. Methods in Enzymology , vol.292
    • Taglicht, D.1    Michaelis, S.2
  • 121
    • 0022534951 scopus 로고
    • A family of related ATP-binding subunits coupled to many distinct biological processes in bacteria
    • 121. Higgins CF, Hiles ID, Salmond GPC, et al. A family of related ATP-binding subunits coupled to many distinct biological processes in bacteria. Nature. 1986;323:448-450.
    • (1986) Nature , vol.323 , pp. 448-450
    • Higgins, C.F.1    Hiles, I.D.2    Salmond, G.P.C.3
  • 122
    • 0029826866 scopus 로고    scopus 로고
    • + channels (ROMK2) to the inhibitory sulfonylurea compound, glibenclamide, is enhanced by co-expression with the ATP-binding cassette transporter CFTR
    • + channels (ROMK2) to the inhibitory sulfonylurea compound, glibenclamide, is enhanced by co-expression with the ATP-binding cassette transporter CFTR. Proc Natl Acad Sci USA. 1996;93:8083-8088.
    • (1996) Proc Natl Acad Sci USA , vol.93 , pp. 8083-8088
    • McNicholas, C.M.1    Guggino, W.B.2    Schwiebert, E.M.3
  • 123
    • 57249114485 scopus 로고    scopus 로고
    • Sulfonylurea sensitivity of ROMK2 is altered by coexpression with full length and truncated forms of CFTR
    • 123. McNicholas CM, Nason MW, Guggino WB, et al. Sulfonylurea sensitivity of ROMK2 is altered by coexpression with full length and truncated forms of CFTR. J Am Soc Neph. 1996;7:1286.
    • (1996) J Am Soc Neph. , vol.7 , pp. 1286
    • McNicholas, C.M.1    Nason, M.W.2    Guggino, W.B.3


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