메뉴 건너뛰기




Volumn 14, Issue 10, 2004, Pages 1465-1483

Pharmacological interventions for the correction of ion transport defect in cystic fibrosis

Author keywords

Amiloride sensitive Na+ channel (ENaC); Cystic fibrosis therapy; Cystic fibrosis transmembrane conductance regulator (CFTR) Cl channel; Epithelial K+ channels; Epithelial secretion; High throughput screening (HTS); Pharmacology; Polypeptides; Small molecules

Indexed keywords

1,3 DIALLYL 8 CYCLOPENTYLXANTHINE; 1,7 DIHYDRO 2 (PROPOXYPHENYL) 6H PURIN 6 ONE; 4 PHENYLBUTYRIC ACID; 8 CYCLOPENTYL 1,3 DIPROPYL 7 METHYLXANTHINE; 8 CYCLOPENTYL 1,3 DIPROPYLXANTHINE; ACETAMIDE DERIVATIVE; ADENOSINE TRIPHOSPHATE; AMINOGLYCOSIDE ANTIBIOTIC AGENT; APIGENIN; ARYLBUTYRIC ACID DERIVATIVE; BUPHENYL; CAFFEINE DERIVATIVE; CALCIUM CHANNEL BLOCKING AGENT; CHLORIDE; CHLORIDE CHANNEL; CURCUMIN; CYCLIC AMP; CYCLOHEXYLCAFFEINE; CYCLOPIAZONIC ACID; FLAVONE DERIVATIVE; GENISTEIN; GENTAMICIN; HALOTHANE; ISOFLAVONE DERIVATIVE; PHOSPHODIESTERASE INHIBITOR; QUINOLIZINE DERIVATIVE; SESQUITERPENE LACTONE DERIVATIVE; SODIUM CHLORIDE; THAPSIGARGIN; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG; UNINDEXED DRUG; XANTHINE DERIVATIVE;

EID: 5744227335     PISSN: 13543776     EISSN: None     Source Type: Journal    
DOI: 10.1517/13543776.14.10.1465     Document Type: Review
Times cited : (6)

References (99)
  • 3
    • 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
  • 4
    • 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. (1986) 78:1245-1252.
    • (1986) J. Clin. Invest. , vol.78 , pp. 1245-1252
    • Boucher, R.C.1    Stutts, M.J.2    Knowles, M.R.3    Cantley, L.4    Gatzy, J.T.5
  • 5
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
    • 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
  • 6
    • 0346636602 scopus 로고    scopus 로고
    • The cystic fibrosis transmembrane conductance regulator (ABCC7)
    • Holland IB, Cole S, Kuchler K, Higgins CF (Eds). Academic Press
    • HANRAHAN JW, GENTZSCH M, RIORDAN JR: The cystic fibrosis transmembrane conductance regulator (ABCC7). In: ABC Proteins: From Bacteria to Man. Holland IB, Cole S, Kuchler K, Higgins CF (Eds). Academic Press (2003):589-618.
    • (2003) ABC Proteins: From Bacteria to Man , pp. 589-618
    • Hanrahan, J.W.1    Gentzsch, M.2    Riordan, J.R.3
  • 9
    • 0032912589 scopus 로고    scopus 로고
    • Structure and function of the CFTR chloride channel
    • SHEPPARD DN, WELSH MJ: Structure and function of the CFTR chloride channel. Physiol. Rev. (1999) 79:23-45.
    • (1999) Physiol. Rev. , vol.79 , pp. 23-45
    • Sheppard, D.N.1    Welsh, M.J.2
  • 11
    • 0032936619 scopus 로고    scopus 로고
    • Contol of CFTR channel gating by phosphorylation and nucleotide hydrolysis
    • GADSBY DC, NAIRN AC: Contol of CFTR channel gating by phosphorylation and nucleotide hydrolysis. Physiol. Rev. (1999) 79:77-107.
    • (1999) Physiol. Rev. , vol.79 , pp. 77-107
    • Gadsby, D.C.1    Nairn, A.C.2
  • 12
    • 0028577602 scopus 로고
    • Phosphatase inhibitors activate normal and defective CFTR chloride channels
    • BECQ F, JENSEN TJ, CHANG X-B et al.: Phosphatase inhibitors activate normal and defective CFTR chloride channels. Proc. Natl. Acad. Sci. USA (1994) 91:9160-9164.
    • (1994) Proc. Natl. Acad. Sci. USA , vol.91 , pp. 9160-9164
    • Becq, F.1    Jensen, T.J.2    Chang, X.-B.3
  • 13
    • 0037460729 scopus 로고    scopus 로고
    • Cystic fibrosis
    • RATJEN F, DORING G: Cystic fibrosis. Lancet (2003) 361:681-689.
    • (2003) Lancet , vol.361 , pp. 681-689
    • Ratjen, F.1    Doring, G.2
  • 14
    • 0027162649 scopus 로고
    • Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
    • WELSH MJ, SMITH AE: Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell (1993) 73 1251-1254.
    • (1993) Cell , vol.73 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 15
    • 0025242929 scopus 로고
    • Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
    • CHENG SH, GREGORY RJ, MARSHALL J et al.: Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell (1990) 63:827-834.
    • (1990) Cell , vol.63 , pp. 827-834
    • Cheng, S.H.1    Gregory, R.J.2    Marshall, J.3
  • 16
    • 0036896021 scopus 로고    scopus 로고
    • Rescuing protein conformation: Prospects for pharmacological therapy in cystic fibrosis
    • GELMAN MS, KOPITO RR: Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis. J. Clin. Invest. (2002) 110:1591-1597.
    • (2002) J. Clin. Invest. , vol.110 , pp. 1591-1597
    • Gelman, M.S.1    Kopito, R.R.2
  • 17
    • 0026649584 scopus 로고
    • The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide
    • THOMAS PJ, SHENBAGAMURTHI P, SONDEK J, HULLIHEN JM, PEDERSEN PL: The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide. J. Biol. Chem. (1992) 267:5727-5730.
    • (1992) J. Biol. Chem. , vol.267 , pp. 5727-5730
    • Thomas, P.J.1    Shenbagamurthi, P.2    Sondek, J.3    Hullihen, J.M.4    Pedersen, P.L.5
  • 18
    • 0028840915 scopus 로고
    • Degradation of CFTR by the ubiquitin-proteasome pathway
    • WARD CL, OMURA S, KOPITO RR: Degradation of CFTR by the ubiquitin-proteasome pathway. Cell (1995) 83:122-127.
    • (1995) Cell , vol.83 , pp. 122-127
    • Ward, C.L.1    Omura, S.2    Kopito, R.R.3
  • 19
    • 0025310336 scopus 로고
    • A cluster of cystic fibrosis mutations in the first nucleotide binding fold domain of the cystic fibrosis conductance regulator protein
    • CUTTING GR, KASCH LM, ROSENSTEIN BJ et al.: A cluster of cystic fibrosis mutations in the first nucleotide binding fold domain of the cystic fibrosis 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
  • 20
    • 0028264035 scopus 로고
    • Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding
    • LOGAN J, HIESTAND D, DARAM P et al.: Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding. J. Clin. Invest. (1994) 94:228-326.
    • (1994) J. Clin. Invest. , vol.94 , pp. 228-326
    • Logan, J.1    Hiestand, D.2    Daram, P.3
  • 21
    • 0037027950 scopus 로고    scopus 로고
    • Therapeutic approaches to repair defects in ΔF508 CFTR folding and cellular targeting
    • POWELL K, ZEITLIN PL: Therapeutic approaches to repair defects in ΔF508 CFTR folding and cellular targeting. Ad. Drug Del. Rev. (2002) 54:1395-1408.
    • (2002) Ad. Drug Del. Rev. , vol.54 , pp. 1395-1408
    • Powell, K.1    Zeitlin, P.L.2
  • 22
    • 0026325533 scopus 로고
    • Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
    • DALEMANS W, BARBRY P, CHAMPIGNY G et al.: Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation. Nature (1991) 354:526-528.
    • (1991) Nature , vol.354 , pp. 526-528
    • Dalemans, W.1    Barbry, P.2    Champigny, G.3
  • 23
    • 0026337305 scopus 로고
    • Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes
    • DRUMM ML, WILKINSON DJ, SMIT LS et al.: Chloride conductance expressed by delta F508 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.S.3
  • 25
    • 0026611093 scopus 로고
    • A1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells
    • EIDELMAN O, GUAY-BRODER C, VAN GALEN PJ et al.: A1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells. Proc. Natl. Acad. Sci. USA (1992) 89 5562-5566.
    • (1992) Proc. Natl. Acad. Sci. USA , vol.89 , pp. 5562-5566
    • Eidelman, O.1    Guay-Broder, C.2    Van Galen, P.J.3
  • 26
    • 0028990824 scopus 로고
    • A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator ΔF508 mutation
    • GUAY-BRODER C, JACOBSON KA, BARNOY ZI et al.: A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator ΔF508 mutation. Biochemistry (1995) 34:9079-9087.
    • (1995) Biochemistry , vol.34 , pp. 9079-9087
    • Guay-Broder, C.1    Jacobson, K.A.2    Barnoy, Z.I.3
  • 27
    • 0029153777 scopus 로고
    • Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors
    • JACOBSON KA, GUAY-BRODER C, VAN GALEN PJ et al.: Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors. Biochemistry (1995) 34 9088-9094.
    • (1995) Biochemistry , vol.34 , pp. 9088-9094
    • Jacobson, K.A.1    Guay-Broder, C.2    Van Galen, P.J.3
  • 28
    • 0032489513 scopus 로고    scopus 로고
    • Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX)
    • ARISPE N, MA J, JACOBSON KA, POLLARD HB: Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX). J. Biol. Chem. (1997) 273:5727-5734.
    • (1997) J. Biol. Chem. , vol.273 , pp. 5727-5734
    • Arispe, N.1    Ma, J.2    Jacobson, K.A.3    Pollard, H.B.4
  • 29
    • 0030943895 scopus 로고    scopus 로고
    • 8-cyclopentyl-1-3-dipropylxanthine and other xanthines differentially bind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator
    • COHEN BE, LEE G, JACOBSON KA et al.: 8-cyclopentyl-1-3-dipropylxanthine and other xanthines differentially bind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator. Biochemistry (1997) 36:6455-6461.
    • (1997) Biochemistry , vol.36 , pp. 6455-6461
    • Cohen, B.E.1    Lee, G.2    Jacobson, K.A.3
  • 30
    • 0031990988 scopus 로고    scopus 로고
    • Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel
    • CHAPPE V, METTEY Y, VIERFOND J-M et al.: Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel. Br. J. Pharmacol. (1998) 123 683-693.
    • (1998) Br. J. Pharmacol. , vol.123 , pp. 683-693
    • Chappe, V.1    Mettey, Y.2    Vierfond, J.-M.3
  • 31
    • 0036161237 scopus 로고    scopus 로고
    • A Phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis
    • McCARTY N, STANDART TA, TERESI M et al.: A Phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis. Ped. Pulm. (2002) 33:90-98.
    • (2002) Ped. Pulm. , vol.33 , pp. 90-98
    • McCarty, N.1    Standart, T.A.2    Teresi, M.3
  • 32
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
    • 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
  • 33
    • 0024596704 scopus 로고
    • Inhibition of heat shock (stess) protein induction by deuterium oxide and glycerol: Additional support for the abnormal protein hypothesis of induction
    • EDINGTON BV, WHELAN SA, HIGHTOWER LE: Inhibition of heat shock (stess) protein induction by deuterium oxide and glycerol: additional support for the abnormal protein hypothesis of induction. J. Cell Physiol. (1989) 139:219-228.
    • (1989) J. Cell Physiol. , vol.139 , pp. 219-228
    • Edington, B.V.1    Whelan, S.A.2    Hightower, L.E.3
  • 34
    • 0030154620 scopus 로고    scopus 로고
    • Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein
    • BROWN CR, HONG-BROWN LQ, BIWERSI J, VERKMAN AS, WELCH WJ: Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein. Cell Stess Chap. (1996) 1:117-125.
    • (1996) Cell Stess Chap. , vol.1 , pp. 117-125
    • Brown, C.R.1    Hong-Brown, L.Q.2    Biwersi, J.3    Verkman, A.S.4    Welch, W.J.5
  • 35
    • 0042317083 scopus 로고    scopus 로고
    • Mammalian osmolytes and S-nitrosoglutathione promote ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function
    • HOWARD M, FISCHER H, ROUX J et al.: Mammalian osmolytes and S-nitrosoglutathione promote ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function. J. Biol. Chem. (2003) 278:35159-35167.
    • (2003) J. Biol. Chem. , vol.278 , pp. 35159-35167
    • Howard, M.1    Fischer, H.2    Roux, J.3
  • 36
    • 0347065355 scopus 로고    scopus 로고
    • Organic solutes rescue the functional defect in ΔF508 cystic fibrosis transmembrane conductance regulator
    • ZHANG XM, WANG XT, YUE H et al.: Organic solutes rescue the functional defect in ΔF508 cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. (2003) 278:51232-51242.
    • (2003) J. Biol. Chem. , vol.278 , pp. 51232-51242
    • Zhang, X.M.1    Wang, X.T.2    Yue, H.3
  • 37
    • 0027536090 scopus 로고
    • Symptomatic and asymptomatic methylenetetrahydrofolate reductase deficiency in two adult brothers
    • HAWORTH JC, DILLING LA, SURTEES RA et al.: Symptomatic and asymptomatic methylenetetrahydrofolate reductase deficiency in two adult brothers. Am. J. Med. Genet. (1993) 45:572-576.
    • (1993) Am. J. Med. Genet. , vol.45 , pp. 572-576
    • Haworth, J.C.1    Dilling, L.A.2    Surtees, R.A.3
  • 38
    • 0028944377 scopus 로고
    • Functional activation of the cystic fibrosis trafficking mutant ΔF508-CFTR by overexpression
    • CHENG SH, FANG SL, ZABNER J et al.: Functional activation of the cystic fibrosis trafficking mutant ΔF508-CFTR by overexpression. Am. J. Physiol. (1995) 268:615-624.
    • (1995) Am. J. Physiol. , vol.268 , pp. 615-624
    • Cheng, S.H.1    Fang, S.L.2    Zabner, J.3
  • 39
    • 0030809817 scopus 로고    scopus 로고
    • In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing ΔF508-CFTR
    • RUBENSTEIN RC, EGAN ME, ZEITLIN PL: In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing ΔF508-CFTR. J. Clin. Invest. (1997) 100:2457-2465.
    • (1997) J. Clin. Invest. , vol.100 , pp. 2457-2465
    • Rubenstein, R.C.1    Egan, M.E.2    Zeitlin, P.L.3
  • 40
    • 0034099743 scopus 로고    scopus 로고
    • Sodium 4-phenylbutyrate downregulates Hsc70: Implications for intracellular trafficking of ΔF508-CFTR
    • RUBENSTEIN RC, ZEITLIN PL: Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of ΔF508-CFTR. Am. J. Phyiol. (2000) 278:259-267.
    • (2000) Am. J. Phyiol. , vol.278 , pp. 259-267
    • Rubenstein, R.C.1    Zeitlin, P.L.2
  • 41
    • 0031889082 scopus 로고    scopus 로고
    • A pilot clinical trial of oral sodium 4-phenylbutyrate (buphenyl) in ΔF508-homozygous cystic fibrosis patients
    • RUBENSTEIN RC, ZEITLIN PA: A pilot clinical trial of oral sodium 4-phenylbutyrate (buphenyl) in ΔF508-homozygous cystic fibrosis patients. Am. J. Respir. Crit. Care Med. (1998) 157:484-490.
    • (1998) Am. J. Respir. Crit. Care Med. , vol.157 , pp. 484-490
    • Rubenstein, R.C.1    Zeitlin, P.A.2
  • 42
    • 0036665609 scopus 로고    scopus 로고
    • Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate
    • ZEITLIN PA, DIENER-WEST M, RUBENSTEIN RC et al.: Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. Mol. Ther. (2002) 6 119-126.
    • (2002) Mol. Ther. , vol.6 , pp. 119-126
    • Zeitlin, P.A.1    Diener-West, M.2    Rubenstein, R.C.3
  • 43
    • 0030702773 scopus 로고    scopus 로고
    • Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line
    • BEDWELL DM, KAENJAK A, BENOS DJ et al.: Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line. Nat. Med. (1997) 3:1280-1284.
    • (1997) Nat. Med. , vol.3 , pp. 1280-1284
    • Bedwell, D.M.1    Kaenjak, A.2    Benos, D.J.3
  • 44
    • 0141863491 scopus 로고    scopus 로고
    • Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations
    • WILCHANSKI M, YAHAV Y, YAACOV Y et al.: Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations. N. Engl. J. Med. (2003) 349 1433-1441.
    • (2003) N. Engl. J. Med. , vol.349 , pp. 1433-1441
    • Wilchanski, M.1    Yahav, Y.2    Yaacov, Y.3
  • 45
    • 0036115199 scopus 로고    scopus 로고
    • Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells
    • EGAN ME, GLOCKNER-PAGEL J, AMBROSE CA et al.: Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells. Nat. Med. (2002) 8:485-492.
    • (2002) Nat. Med. , vol.8 , pp. 485-492
    • Egan, M.E.1    Glockner-Pagel, J.2    Ambrose, C.A.3
  • 46
    • 11144355340 scopus 로고    scopus 로고
    • Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects
    • EGAN ME, PEARSON M, WEINER SA et al.: Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects. Science (2004) 304:600-602.
    • (2004) Science , vol.304 , pp. 600-602
    • Egan, M.E.1    Pearson, M.2    Weiner, S.A.3
  • 47
    • 0026050850 scopus 로고
    • Thapsigargin inhibits the sarcoplasmic or endoplasmi reticulum Ca-ATPase family of calcium pumps
    • LYTTON J, WESTLIN M, HANLEY MR: Thapsigargin inhibits the sarcoplasmic or endoplasmi reticulum Ca-ATPase family of calcium pumps. J. Biol. Chem. (1991) 266:17067-17071.
    • (1991) J. Biol. Chem. , vol.266 , pp. 17067-17071
    • Lytton, J.1    Westlin, M.2    Hanley, M.R.3
  • 48
    • 0033600763 scopus 로고    scopus 로고
    • Development of substituted benzo[c]quinolizinium compounds as novel activators of the cystic cibrosis chloride channel
    • BECQ F, METTEY Y, GRAY MA et al.: Development of substituted benzo[c]quinolizinium compounds as novel activators of the cystic cibrosis chloride channel. J. Biol. Chem. (1999) 274 27415-27425.
    • (1999) J. Biol. Chem. , vol.274 , pp. 27415-27425
    • Becq, F.1    Mettey, Y.2    Gray, M.A.3
  • 49
    • 10744229649 scopus 로고    scopus 로고
    • Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels
    • MARIVINGT-MOUNIR C, NOREZ C, DERAND R et al.: Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels. J. Med. Chem. (2004) 47:962-972.
    • (2004) J. Med. Chem. , vol.47 , pp. 962-972
    • Marivingt-Mounir, C.1    Norez, C.2    Derand, R.3
  • 50
    • 0035195682 scopus 로고    scopus 로고
    • Activation of G551D CFTR channel with MPB-91: Regulation by ATPase activity and phosphorylation
    • DERAND R, BULTEAU-PIGNOUX L, METTEY Y et al.: Activation of G551D CFTR channel with MPB-91: regulation by ATPase activity and phosphorylation. Am. J. Physiol. (2001) 281 1657-1666.
    • (2001) Am. J. Physiol. , vol.281 , pp. 1657-1666
    • Derand, R.1    Bulteau-Pignoux, L.2    Mettey, Y.3
  • 51
    • 0035203949 scopus 로고    scopus 로고
    • Correction of delF508-CFTR activity with benzoquinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells
    • DORMER R L, DERAND R, MCNEILLY C et al.: Correction of delF508-CFTR activity with benzoquinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells. J. Cell Sci. (2001) 114:4073-4081.
    • (2001) J. Cell Sci. , vol.114 , pp. 4073-4081
    • Dormer, R.L.1    Derand, R.2    McNeilly, C.3
  • 53
    • 0033429693 scopus 로고    scopus 로고
    • A cyclic nucleotide PDE5 inhibitor corrects defective mucin secretion in submandibular cells containing antibody directed against the cystic fibrosis transmembrane conductance regulator protein
    • MCPHERSON MA, PEREIRA MM, LLOYD-MILLS C, MURRAY KJ, DORMER RL: A cyclic nucleotide PDE5 inhibitor corrects defective mucin secretion in submandibular cells containing antibody directed against the cystic fibrosis transmembrane conductance regulator protein. FEBS Lett. (1999) 464:48-52.
    • (1999) FEBS Lett. , vol.464 , pp. 48-52
    • Mcpherson, M.A.1    Pereira, M.M.2    Lloyd-Mills, C.3    Murray, K.J.4    Dormer, R.L.5
  • 55
    • 0030041905 scopus 로고    scopus 로고
    • Alternate stimulation of apical CFTR by genistein in epithelia
    • ILLEK B, FISCHER H, MACHEN T: Alternate stimulation of apical CFTR by genistein in epithelia. Am. J. Physiol. (1996) 270 265-275.
    • (1996) Am. J. Physiol. , vol.270 , pp. 265-275
    • Illek, B.1    Fischer, H.2    Machen, T.3
  • 56
    • 0037183975 scopus 로고    scopus 로고
    • The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory genistein binding site
    • DERAND R, BULTEAU-PIGNOUX L, BECQ F: The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory genistein binding site. J. Biol. Chem. (2002) 277 35999-36004.
    • (2002) J. Biol. Chem. , vol.277 , pp. 35999-36004
    • Derand, R.1    Bulteau-Pignoux, L.2    Becq, F.3
  • 57
    • 2442543305 scopus 로고    scopus 로고
    • The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein
    • MELIN P, THOREAU V, NOREZ C, BILAN F, KITZIS A, BECQ F: The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein. Biochem. Pharmacol. (2004) 67:2187-2196.
    • (2004) Biochem. Pharmacol. , vol.67 , pp. 2187-2196
    • Melin, P.1    Thoreau, V.2    Norez, C.3    Bilan, F.4    Kitzis, A.5    Becq, F.6
  • 58
    • 0041589194 scopus 로고    scopus 로고
    • Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: Towards a pharmacophore model for the nucleotide-binding domain
    • SPRINGSTEEL MF, GALIETTA LJ, MAT et al.: Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain. Bio. Med. Chem. (2003) 11:4113-4120.
    • (2003) Bio. Med. Chem. , vol.11 , pp. 4113-4120
    • Springsteel, M.F.1    Galietta, L.J.2    Mat, A.3
  • 59
    • 17044448151 scopus 로고    scopus 로고
    • CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds
    • CACI E, FOLLI C, ZEGARRA-MORAN O et al.: CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds. Am. J. Physiol. (2003) 285 180-188.
    • (2003) Am. J. Physiol. , vol.285 , pp. 180-188
    • Caci, E.1    Folli, C.2    Zegarra-Moran, O.3
  • 60
    • 0034750942 scopus 로고    scopus 로고
    • Phenanthrolines - A new class of CFTR chloride channel openers
    • DUSZYK M, MACVINISH L, CUTHBERT AW: Phenanthrolines - a new class of CFTR chloride channel openers. Br. J. Pharmacol. (2001) 134:853-864.
    • (2001) Br. J. Pharmacol. , vol.134 , pp. 853-864
    • Duszyk, M.1    Macvinish, L.2    Cuthbert, A.W.3
  • 61
    • 0038630416 scopus 로고    scopus 로고
    • Benzoquinolines and chloride secretion in routine colonic epithelium
    • CUTHBERT AW: Benzoquinolines and chloride secretion in routine colonic epithelium. Br. J. Pharmacol. (2003) 138 1528-1534.
    • (2003) Br. J. Pharmacol. , vol.138 , pp. 1528-1534
    • Cuthbert, A.W.1
  • 62
    • 0041769470 scopus 로고    scopus 로고
    • Mechanisms of anion secretion in Calu-3 human airway epithelial cells by 7,8-benzoquinoline
    • CUTHBERT AW, MACVINISH LJ: Mechanisms of anion secretion in Calu-3 human airway epithelial cells by 7,8-benzoquinoline. Br. J. Pharmacol. (2003) 140:81-90.
    • (2003) Br. J. Pharmacol. , vol.140 , pp. 81-90
    • Cuthbert, A.W.1    Macvinish, L.J.2
  • 63
    • 3042700158 scopus 로고    scopus 로고
    • 4-chlorobenzo[F]isoquinoline (CBIQ) activates CFTR chloride channels KCNN4 potassium channels in Calu-3 human airway epithelial cells
    • SZKOTAK AJ, MURTHY M, McVINISH et al.: 4-chlorobenzo[F]isoquinoline (CBIQ) activates CFTR chloride channels and KCNN4 potassium channels in Calu-3 human airway epithelial cells. Br.J. Pharmacol. (2004) 142:531-542.
    • (2004) Br. J. Pharmacol. , vol.142 , pp. 531-542
    • Szkotak, A.J.1    Murthy, M.2    McVinish, A.3
  • 64
    • 0035827680 scopus 로고    scopus 로고
    • Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds
    • GALIETTA LJV, SPRINGSTEEL MF, EDA M et al.: Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds. J. Biol. Chem. (2001) 276:19723-19728.
    • (2001) J. Biol. Chem. , vol.276 , pp. 19723-19728
    • Galietta, L.J.V.1    Springsteel, M.F.2    Eda, M.3
  • 65
    • 0037020097 scopus 로고    scopus 로고
    • High-affiniry activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening
    • MA T, VETRIVEL L, YANG H et al.: High-affiniry activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening. J Biol. Chem. (2002) 277:37235-37241.
    • (2002) J Biol. Chem. , vol.277 , pp. 37235-37241
    • Ma, T.1    Vetrivel, L.2    Yang, H.3
  • 66
    • 0042317111 scopus 로고    scopus 로고
    • Nanomolar affinity small molecule correctors of defective ΔF508-CFTR chloride channel gating
    • YANG H, SHELAT AA, GUY RK et al.: Nanomolar affinity small molecule correctors of defective ΔF508-CFTR chloride channel gating. J. Biol. Chem. (2003) 278:35079-35085.
    • (2003) J. Biol. Chem. , vol.278 , pp. 35079-35085
    • Yang, H.1    Shelat, A.A.2    Guy, R.K.3
  • 67
  • 70
    • 0029853667 scopus 로고    scopus 로고
    • - secretion by benzimidazolones. II. Coordinate regulation of apical GCl and basolateral GK
    • - secretion by benzimidazolones. II. Coordinate regulation of apical GCl and basolateral GK. Am. J. Physiol. (1996) 271:785-795.
    • (1996) Am. J. Physiol. , vol.271 , pp. 785-795
    • Devor, D.C.1    Singh, A.K.2    Bridges, R.J.3    Frizzell, R.A.4
  • 71
    • 0025345720 scopus 로고
    • + channels in airway epithelia. II. Role in Cl secretion evidence for two types of K channels
    • + channels in airway epithelia. II. Role in Cl secretion and evidence for two types of K channels. Am. J. Physiol. (1990) 258:343-348.
    • (1990) Am. J. Physiol. , vol.258 , pp. 343-348
    • McCann, J.D.1    Welsh, M.J.2
  • 73
    • 0344196939 scopus 로고    scopus 로고
    • Modulation of calcium-dependent chloride secretion by basolateral SK4-like channels in a human bronchial cell line
    • BERNARD K, BOGLIOLO S, SORIANI O, EHRENFELD J: Modulation of calcium-dependent chloride secretion by basolateral SK4-like channels in a human bronchial cell line. J. Membr. Biol. (2003) 196 15-31.
    • (2003) J. Membr. Biol. , vol.196 , pp. 15-31
    • Bernard, K.1    Bogliolo, S.2    Soriani, O.3    Ehrenfeld, J.4
  • 74
    • 0037383908 scopus 로고    scopus 로고
    • + channels in human normal and cystic fibrosis airway epithelia
    • + channels in human normal and cystic fibrosis airway epithelia. Pediatr. Res. (2003) 53:608-618.
    • (2003) Pediatr. Res. , vol.53 , pp. 608-618
    • Mall, M.1    Gonska, T.2    Thomas, J.3
  • 76
    • 0030987549 scopus 로고    scopus 로고
    • hSK4, a member of a novel subfamily of calcium-activated potassium channels
    • JOINER WJ, WANG LY, TANG MD, KACZMAREK LK: hSK4, a member of a novel subfamily of calcium-activated potassium channels. Proc. Natl. Acad. Sci. USA (1997) 94:11013-11018.
    • (1997) Proc. Natl. Acad. Sci. USA , vol.94 , pp. 11013-11018
    • Joiner, W.J.1    Wang, L.Y.2    Tang, M.D.3    Kaczmarek, L.K.4
  • 78
    • 0035141190 scopus 로고    scopus 로고
    • A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazolone analogues
    • AL-NAKKASH L, HU S, LI M, HWANG T-C: A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazolone analogues. J. Pharm. Exp. Ther. (2001) 296:464-472.
    • (2001) J. Pharm. Exp. Ther. , vol.296 , pp. 464-472
    • Al-Nakkash, L.1    Hu, S.2    Li, M.3    Hwang, T.-C.4
  • 80
    • 17144453452 scopus 로고    scopus 로고
    • Comparative pharmacology of the activity of wild-type and G551D mutated CFTR chloride channel: Effect of the benzimidazolone derivative NS004
    • DERAND R, BULTEAU-PIGNOUX L, BECQ F: Comparative pharmacology of the activity of wild-type and G551D mutated CFTR chloride channel: effect of the benzimidazolone derivative NS004. J. Membr. Biol. (2003) 194:109-117.
    • (2003) J. Membr. Biol. , vol.194 , pp. 109-117
    • Derand, R.1    Bulteau-Pignoux, L.2    Becq, F.3
  • 81
    • 0035146762 scopus 로고    scopus 로고
    • Benzimidazolone activators of chloride secretion: Potential therapeutics for cystic fibrosis and chronic obstructive pulmonary disease
    • SINGH S, SYME CA, SINGH AK, DEVOR DC, BRIDGES RJ: Benzimidazolone activators of chloride secretion: potential therapeutics for cystic fibrosis and chronic obstructive pulmonary disease. J. Pharm. Exp. Ther. (2001) 296:600-611.
    • (2001) J. Pharm. Exp. Ther. , vol.296 , pp. 600-611
    • Singh, S.1    Syme, C.A.2    Singh, A.K.3    Devor, D.C.4    Bridges, R.J.5
  • 83
    • 0027486593 scopus 로고
    • No added benefit from nebulized amiloride in patients with cystic fibrosis
    • GRAHAM A, HASANI A, ALTON EW et al.: No added benefit from nebulized amiloride in patients with cystic fibrosis. Eur. Respir. J. (1993) 6:1243-1248.
    • (1993) Eur. Respir. J. , vol.6 , pp. 1243-1248
    • Graham, A.1    Hasani, A.2    Alton, E.W.3
  • 84
    • 0034047238 scopus 로고    scopus 로고
    • French multicenter randomised double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients
    • PONS G, MARCHAND MC, D'ATHIS P et al.: French multicenter randomised double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. Pediatr. Pulmonol. (2000) 30:25-31.
    • (2000) Pediatr. Pulmonol. , vol.30 , pp. 25-31
    • Pons, G.1    Marchand, M.C.2    D'Athis, P.3
  • 85
    • 0032833866 scopus 로고    scopus 로고
    • The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis
    • RODGERS HC, KNOX AJ: The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis. Eur. J. Respir. (1999) 14:693-696.
    • (1999) Eur. J. Respir. , vol.14 , pp. 693-696
    • Rodgers, H.C.1    Knox, A.J.2
  • 87
    • 0029785898 scopus 로고    scopus 로고
    • Apical and basolateral ATP stimulate tracheal epithelial chloride secretion via multiple purinergic receptors and signalling pathways
    • HWANG TH, SCHWIEBERT EM, GUGGINO WB: Apical and basolateral ATP stimulate tracheal epithelial chloride secretion via multiple purinergic receptors and signalling pathways. Am. J. Physiol. (1996) 270:1611-1623.
    • (1996) Am. J. Physiol. , vol.270 , pp. 1611-1623
    • Hwang, T.H.1    Schwiebert, E.M.2    Guggino, W.B.3
  • 88
    • 0028047286 scopus 로고
    • Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice
    • CLARK LL, GRUBB BR, YANKASKAS JR, COTTON CU, McKENZIE A, BOUCHER RC: Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice. Proc. Natl. Acad. Sci. USA (1994) 91 479-483.
    • (1994) Proc. Natl. Acad. Sci. USA , vol.91 , pp. 479-483
    • Clark, L.L.1    Grubb, B.R.2    Yankaskas, J.R.3    Cotton, C.U.4    McKenzie, A.5    Boucher, R.C.6
  • 89
    • 0038016550 scopus 로고    scopus 로고
    • Antagonists of myo-Inositol 3,4,5,6-tetrakisphophate allow repeated epithelial chloride secretion
    • RUDOLF MT, DINKEL C, TRAYNOR-KAPLAN AE, SCHULTZ C: Antagonists of myo-Inositol 3,4,5,6-tetrakisphophate allow repeated epithelial chloride secretion. Bioorg. Med. Chem. (2003) 11 3315-3329.
    • (2003) Bioorg. Med. Chem. , vol.11 , pp. 3315-3329
    • Rudolf, M.T.1    Dinkel, C.2    Traynor-Kaplan, A.E.3    Schultz, C.4
  • 90
    • 0028032671 scopus 로고
    • Long-term uncoupling ofchloride secretion from intracellular calcium levels by Ins(3,4,5,6)P4
    • VAJANAPHANICH M, SCHULTZ C, RUDOLF MT et al.: Long-term uncoupling ofchloride secretion from intracellular calcium levels by Ins(3,4,5,6)P4. Nature (1994) 371:711-714.
    • (1994) Nature , vol.371 , pp. 711-714
    • Vajanaphanich, M.1    Schultz, C.2    Rudolf, M.T.3
  • 92
    • 1642493889 scopus 로고    scopus 로고
    • A Phase I trial of intranasal Moli1901 for cystic fibrosis
    • ZEITLIN PL, BOYLE MP, GUGGINO WB, MOLINA L: A Phase I trial of intranasal Moli1901 for cystic fibrosis. Chest (2004) 125 143-149.
    • (2004) Chest , vol.125 , pp. 143-149
    • Zeitlin, P.L.1    Boyle, M.P.2    Guggino, W.B.3    Molina, L.4
  • 93
    • 0033166350 scopus 로고    scopus 로고
    • Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis
    • CHANG XB, CUI L, HOU YX et al.: Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis. Mol. Cell. (1999) 4:137-142.
    • (1999) Mol. Cell , vol.4 , pp. 137-142
    • Chang, X.B.1    Cui, L.2    Hou, Y.X.3
  • 94
    • 0142184471 scopus 로고    scopus 로고
    • Rescue of functional D508-CFTR channels by co-expression with truncated CFTR constructs in COS-1 cells
    • OWSIANIK G, CAO L, NILIUS B: Rescue of functional D508-CFTR channels by co-expression with truncated CFTR constructs in COS-1 cells. FEBS Lett (2003) 554:173-178.
    • (2003) FEBS Lett. , vol.554 , pp. 173-178
    • Owsianik, G.1    Cao, L.2    Nilius, B.3
  • 95
    • 0033103174 scopus 로고    scopus 로고
    • A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane K(ATP) channels
    • ZERANGUE N, SCHWAPPACH B, JAN YN, JAN LY: A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane K(ATP) channels. Neuron (1999) 22:537-548.
    • (1999) Neuron , vol.22 , pp. 537-548
    • Zerangue, N.1    Schwappach, B.2    Jan, Y.N.3    Jan, L.Y.4
  • 96
    • 0037151094 scopus 로고    scopus 로고
    • A short segment of the R domain of cystic fibrosis transmembrane conductance regulator contains channel stimulatory and inhibitory activities that are separable by sequence modification
    • XIE J, ADAM LM, ZHAO J, GERKEN TA, DAVIS PB, MA J: A short segment of the R domain of cystic fibrosis transmembrane conductance regulator contains channel stimulatory and inhibitory activities that are separable by sequence modification. J. Biol. Chem (2002) 277 23019-23027.
    • (2002) J. Biol. Chem , vol.277 , pp. 23019-23027
    • Xie, J.1    Adam, L.M.2    Zhao, J.3    Gerken, T.A.4    Davis, P.B.5    Ma, J.6
  • 97
    • 1442349808 scopus 로고    scopus 로고
    • Drug discovery in academia
    • VERKMAN AS: Drug discovery in academia. Am. J. Physiol. (2004) 286:465-474.
    • (2004) Am. J. Physiol. , vol.286 , pp. 465-474
    • Verkman, A.S.1
  • 98
    • 0034330457 scopus 로고    scopus 로고
    • Traffic jam: A compendium of human diseases that affect intracellular transport processes
    • ARIDOR M, HANNAN LA: Traffic jam: A compendium of human diseases that affect intracellular transport processes. Traffic (2000) 1:836-851.
    • (2000) Traffic , vol.1 , pp. 836-851
    • Aridor, M.1    Hannan, L.A.2
  • 99
    • 0036843129 scopus 로고    scopus 로고
    • Traffic jam II: An update of diseases of intracellular transport
    • ARIDOR M, HANNAN LA: Traffic jam II: An update of diseases of intracellular transport. Traffic (2002) 3:781-790.
    • (2002) Traffic , vol.3 , pp. 781-790
    • Aridor, M.1    Hannan, L.A.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.