-
3
-
-
0027958441
-
+ 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
-
+ 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
-
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
-
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
-
8
-
-
0242593119
-
Structure of ABC transporters
-
Holland IB, Cole S, Kuchler K, Higgins CF (Eds) Academic Press
-
LINTON KJ, ROSENBERG MF, KERR ID, HIGGINS CF: Structure of ABC transporters. In: ABC Proteins: From Bacteria to Man. Holland IB, Cole S, Kuchler K, Higgins CF (Eds) Academic Press (2003):65-80.
-
(2003)
ABC Proteins: From Bacteria to Man
, pp. 65-80
-
-
Linton, K.J.1
Rosenberg, M.F.2
Kerr, I.D.3
Higgins, C.F.4
-
9
-
-
0032912589
-
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
-
10
-
-
0032956071
-
CFTR is a conductance regulator as well as a chloride channel
-
SCHWIEBERT EM, BENOS DJ, EGAN ME, STUTTS J, GUGGINO WB: CFTR is a conductance regulator as well as a chloride channel. Physiol. Rev. (1999) 79:145-166.
-
(1999)
Physiol. Rev.
, vol.79
, pp. 145-166
-
-
Schwiebert, E.M.1
Benos, D.J.2
Egan, M.E.3
Stutts, J.4
Guggino, W.B.5
-
11
-
-
0032936619
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
52
-
-
0037427513
-
Benzo(c)quinolizinium drugs inhibit degradation of ΔF508-CFTR cytoplamsic domain
-
STRATFORD FL, PEREIRA MM, BECQ F, MCPHERSON MA, DORMER RL: Benzo(c)quinolizinium drugs inhibit degradation of ΔF508-CFTR cytoplamsic domain. Biochem. Biophys. Res. Commun. (2003) 300 524-530.
-
(2003)
Biochem. Biophys. Res. Commun.
, vol.300
, pp. 524-530
-
-
Stratford, F.L.1
Pereira, M.M.2
Becq, F.3
McPherson, M.A.4
Dormer, R.L.5
-
53
-
-
0033429693
-
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
-
54
-
-
0028928015
-
cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein
-
ILLEK B, FISCHER H, SANTOS GF, WIDDICOMBE JH, MACHEN TE, REENSTRA WW: cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein. Am. J. Physiol. (1995) 268 886-893.
-
(1995)
Am. J. Physiol.
, vol.268
, pp. 886-893
-
-
Illek, B.1
Fischer, H.2
Santos, G.F.3
Widdicombe, J.H.4
Machen, T.E.5
Reenstra, W.W.6
-
55
-
-
0030041905
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
-
0037971989
-
3-(2-benzyphenyl)isoxazoles and isoxazolines: Synthesis and evaluation as CFTR activators
-
SAMMELSON RE, MA T, GALIETTA LJV, VERKMAN AS, KURTH MJ: 3-(2-benzyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators. Bio. Med. Chem. Lett. (2003) 13:2509-2512.
-
(2003)
Bio. Med. Chem. Lett.
, vol.13
, pp. 2509-2512
-
-
Sammelson, R.E.1
Ma, T.2
Galietta, L.J.V.3
Verkman, A.S.4
Kurth, M.J.5
-
71
-
-
0025345720
-
+ 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
-
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
-
+ 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
-
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
-
77
-
-
0028247368
-
The substituted benzimidazolone NS004 is an opener of the cystic fibrosis chloride channel
-
GRIBKOFF VK, CHAMPIGNY G, BABRY P, DWORETZKY SI, MEANWELL NA, LAZDUNSKI M: The substituted benzimidazolone NS004 is an opener of the cystic fibrosis chloride channel. J. Biol. Chem. (1994) 269 10983-10986.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 10983-10986
-
-
Gribkoff, V.K.1
Champigny, G.2
Babry, P.3
Dworetzky, S.I.4
Meanwell, N.A.5
Lazdunski, M.6
-
78
-
-
0035141190
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
-
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
|