-
1
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., Zielinski J., Lok S., Plavsic N., Chou J.L. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 245:1989;1066-1073.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielinski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
2
-
-
0028840915
-
Degradation of CFTR by the ubiquitin-proteasome pathway
-
Ward C.L., Omura S., Kopito R.R. Degradation of CFTR by the ubiquitin-proteasome pathway. Cell. 83:1995;121-127.
-
(1995)
Cell
, vol.83
, pp. 121-127
-
-
Ward, C.L.1
Omura, S.2
Kopito, R.R.3
-
3
-
-
0028559511
-
Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP
-
Lukacs G.L., Mohamed A., Kartner N., Chang X.B., Riordan J.R., Grinstein S. Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP. EMBO J. 13:1994;6076-6086.
-
(1994)
EMBO J.
, vol.13
, pp. 6076-6086
-
-
Lukacs, G.L.1
Mohamed, A.2
Kartner, N.3
Chang, X.B.4
Riordan, J.R.5
Grinstein, S.6
-
4
-
-
0028006681
-
Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins
-
Ward C.L., Kopito R.R. Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins. J. Biol. Chem. 269:1994;25710-25718.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 25710-25718
-
-
Ward, C.L.1
Kopito, R.R.2
-
5
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
Cheng S.H., Gregory R.J., Marshall J., Paul S., Souza D.W., White G.A., O'Riordan A.E. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell. 63:1990;827-834.
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
Paul, S.4
Souza, D.W.5
White, G.A.6
O'Riordan, A.E.7
-
6
-
-
0032588980
-
Delta F508 CFTR protein expression in tissues from patients with cystic fibrosis
-
Kälin N., Claass A., Sommer M., Puchelle E., Tümmler B. Delta F508 CFTR protein expression in tissues from patients with cystic fibrosis. J. Clin. Invest. 103:1999;1379-1389.
-
(1999)
J. Clin. Invest.
, vol.103
, pp. 1379-1389
-
-
Kälin, N.1
Claass, A.2
Sommer, M.3
Puchelle, E.4
Tümmler, B.5
-
7
-
-
0033918281
-
Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells
-
Penque D., Mendes F., Beck S., Farinha C., Pacheco P., Nogueira P., Lavinha J., Malho R., Amaral M.D. Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells. Lab. Invest. 80:2000;857-868.
-
(2000)
Lab. Invest.
, vol.80
, pp. 857-868
-
-
Penque, D.1
Mendes, F.2
Beck, S.3
Farinha, C.4
Pacheco, P.5
Nogueira, P.6
Lavinha, J.7
Malho, R.8
Amaral, M.D.9
-
8
-
-
0037106481
-
The Hdj-1/Hsp40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70
-
Farinha C.M., Nogueira P., Mendes F., Penque D., Amaral M.D. The Hdj-1/Hsp40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70. Biochem. J. 366:2002;797-806.
-
(2002)
Biochem. J.
, vol.366
, pp. 797-806
-
-
Farinha, C.M.1
Nogueira, P.2
Mendes, F.3
Penque, D.4
Amaral, M.D.5
-
9
-
-
0033559258
-
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis
-
Meacham G.C., Lu Z., King S., Sorscher E., Tousson A., Cyr D.M. The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis. EMBO J. 18:1999;1492-1505.
-
(1999)
EMBO J.
, vol.18
, pp. 1492-1505
-
-
Meacham, G.C.1
Lu, Z.2
King, S.3
Sorscher, E.4
Tousson, A.5
Cyr, D.M.6
-
10
-
-
0028232167
-
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator
-
Pind S., Riordan J.R., Williams D.B. Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269:1994;12784-12788.
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 12784-12788
-
-
Pind, S.1
Riordan, J.R.2
Williams, D.B.3
-
11
-
-
0027488993
-
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
-
Yang Y., Janich S., Cohn J.A., Wilson J.M. The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment. Proc. Natl. Acad. Sci. USA. 90:1993;9480-9484.
-
(1993)
Proc. Natl. Acad. Sci. USA
, vol.90
, pp. 9480-9484
-
-
Yang, Y.1
Janich, S.2
Cohn, J.A.3
Wilson, J.M.4
-
12
-
-
0030798979
-
The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator
-
Strickland E., Qu B.H., Millen L., Thomas P.J. The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 272:1997;25421-25424.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 25421-25424
-
-
Strickland, E.1
Qu, B.H.2
Millen, L.3
Thomas, P.J.4
-
13
-
-
0035142877
-
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation
-
Meacham G.C., Patterson C., Zhang W., Younger J.M., Cyr D.M. The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation. Nat. Cell Biol. 3:2001;100-105.
-
(2001)
Nat. Cell Biol.
, vol.3
, pp. 100-105
-
-
Meacham, G.C.1
Patterson, C.2
Zhang, W.3
Younger, J.M.4
Cyr, D.M.5
-
14
-
-
0031004769
-
Ubiquitin-dependent degradation of certain protein substrates in vitro requires the molecular chaperone Hsc70
-
Bercovich B., Stancovski I., Mayer A., Blumenfeld N., Laszlo A., Schwarts A.L., Ciechanover A. Ubiquitin-dependent degradation of certain protein substrates in vitro requires the molecular chaperone Hsc70. J. Biol. Chem. 272:1997;9002-9010.
-
(1997)
J. Biol. Chem.
, vol.272
, pp. 9002-9010
-
-
Bercovich, B.1
Stancovski, I.2
Mayer, A.3
Blumenfeld, N.4
Laszlo, A.5
Schwarts, A.L.6
Ciechanover, A.7
-
15
-
-
0034099743
-
Sodium 4-phenylbutyrate downregulates Hsc70: Implications for intracellular trafficking of DeltaF508-CFTR
-
Rubenstein R.C., Zeitlin P.L. Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR. Am. J. Physiol. Cell Physiol. 278:2000;C259-C267.
-
(2000)
Am. J. Physiol. Cell Physiol.
, vol.278
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
16
-
-
0034805392
-
Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation
-
Rubenstein R.C., Lyons B.M. Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation. Am. J. Physiol. Lung Cell. Mol. Physiol. 281:2001;L43-L51.
-
(2001)
Am. J. Physiol. Lung Cell. Mol. Physiol.
, vol.281
-
-
Rubenstein, R.C.1
Lyons, B.M.2
-
18
-
-
0033562963
-
Inhibition of ATPase, GTPase and adenylate kinase activities of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator by genistein
-
Randak C., Auerswald E.A., Assfalg-Machleidt I., Reenstra W.W., Machleidt W. Inhibition of ATPase, GTPase and adenylate kinase activities of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator by genistein. Biochem. J. 340:1999;227-235.
-
(1999)
Biochem. J.
, vol.340
, pp. 227-235
-
-
Randak, C.1
Auerswald, E.A.2
Assfalg-Machleidt, I.3
Reenstra, W.W.4
Machleidt, W.5
-
19
-
-
0035141190
-
A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazole analogs
-
Al Nakkash L., Hu S., Li M., Hwang T.C. A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazole analogs. J. Pharmacol. Exp. Ther. 296:2001;464-472.
-
(2001)
J. Pharmacol. Exp. Ther.
, vol.296
, pp. 464-472
-
-
Al Nakkash, L.1
Hu, S.2
Li, M.3
Hwang, T.C.4
-
20
-
-
0031881489
-
Action of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects
-
Wang F., Zeltwanger S., Yang I.C., Nairn A.C., Hwang T.C. Action of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects. J. Gen. Physiol. 111:1998;477-490.
-
(1998)
J. Gen. Physiol.
, vol.111
, pp. 477-490
-
-
Wang, F.1
Zeltwanger, S.2
Yang, I.C.3
Nairn, A.C.4
Hwang, T.C.5
-
21
-
-
0033625415
-
Activation of delta F508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX
-
Andersson C., Roomans G.M. Activation of delta F508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX. Eur. Respir. J. 15:2000;937-941.
-
(2000)
Eur. Respir. J.
, vol.15
, pp. 937-941
-
-
Andersson, C.1
Roomans, G.M.2
-
22
-
-
0028292615
-
CFTR in Calu-3 human airway cells: Channel properties and role in cAMP-activated Cl-conductance
-
Haws C., Finkbeiner W.E., Widdicombe J.H., Wine J.J. CFTR in Calu-3 human airway cells: channel properties and role in cAMP-activated Cl-conductance. Am. J. Physiol. 266:1994;L502-L512.
-
(1994)
Am. J. Physiol.
, vol.266
-
-
Haws, C.1
Finkbeiner, W.E.2
Widdicombe, J.H.3
Wine, J.J.4
-
23
-
-
0025032271
-
Development and biological applications of chloride-sensitive fluorescent indicators
-
Verkman A.S. Development and biological applications of chloride-sensitive fluorescent indicators. Am. J. Physiol. 259:1990;C375-C388.
-
(1990)
Am. J. Physiol.
, vol.259
-
-
Verkman, A.S.1
-
24
-
-
0025020683
-
Chloride conductive and cotransport mechanisms in cultures of canine tracheal epithelial cells measured by an entrapped fluorescent indicator
-
Chao A.C., Widdicombe J.H., Verkman A.S. Chloride conductive and cotransport mechanisms in cultures of canine tracheal epithelial cells measured by an entrapped fluorescent indicator. J. Membr. Biol. 113:1990;193-202.
-
(1990)
J. Membr. Biol.
, vol.113
, pp. 193-202
-
-
Chao, A.C.1
Widdicombe, J.H.2
Verkman, A.S.3
-
25
-
-
0033864526
-
Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia
-
Devor D.C., Bridges R.J., Pilewski J.M. Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia. Am. J. Physiol. Cell Physiol. 279:2000;C461-C479.
-
(2000)
Am. J. Physiol. Cell Physiol.
, vol.279
-
-
Devor, D.C.1
Bridges, R.J.2
Pilewski, J.M.3
-
26
-
-
0033838554
-
Effect of genistein on native epithelial tissue from normal individuals and CF patients and on ion channels expressed in Xenopus oocytes
-
Mall M., Wissner A., Seydewitz H.H., Hubner M., Kuehr J., Brandis M., Kunzelmann K. Effect of genistein on native epithelial tissue from normal individuals and CF patients and on ion channels expressed in Xenopus oocytes. Br. J. Pharmacol. 130:2000;1884-1892.
-
(2000)
Br. J. Pharmacol.
, vol.130
, pp. 1884-1892
-
-
Mall, M.1
Wissner, A.2
Seydewitz, H.H.3
Hubner, M.4
Kuehr, J.5
Brandis, M.6
Kunzelmann, K.7
-
27
-
-
0029202027
-
Genistein and tyrphostin 47 stimulate CFTR-mediated Cl-secretion in T84 cell monolayers
-
Sears C.L., Firoozmand F., Mellander A., Chambers F.G., Eromar I.G., Bot A.G., Scholte B., de Jonge H.R., Donowitz M. Genistein and tyrphostin 47 stimulate CFTR-mediated Cl-secretion in T84 cell monolayers. Am. J. Physiol. 269:1995;G874-G882.
-
(1995)
Am. J. Physiol.
, vol.269
-
-
Sears, C.L.1
Firoozmand, F.2
Mellander, A.3
Chambers, F.G.4
Eromar, I.G.5
Bot, A.G.6
Scholte, B.7
De Jonge, H.R.8
Donowitz, M.9
-
28
-
-
0030041905
-
Alternative stimulation of apical CFTR by genistein in epithelia
-
Illek B., Fisher H., Machen T.E. Alternative stimulation of apical CFTR by genistein in epithelia. Am. J. Physiol. 270:1996;C265-C275.
-
(1996)
Am. J. Physiol.
, vol.270
-
-
Illek, B.1
Fisher, H.2
Machen, T.E.3
-
29
-
-
0028343434
-
Daidzein is a more bioavailable soymilk isoflavone than is genistein in adult women
-
Xu X., Wang H.J., Murphy P.A., Cook L., Hendrich S. Daidzein is a more bioavailable soymilk isoflavone than is genistein in adult women. J. Nutr. 124:1994;825-832.
-
(1994)
J. Nutr.
, vol.124
, pp. 825-832
-
-
Xu, X.1
Wang, H.J.2
Murphy, P.A.3
Cook, L.4
Hendrich, S.5
-
30
-
-
0030773897
-
Genistein potentiates wild-type and delta F508-CFTR channel activity
-
Hwang T.C., Wang F., Yang I.C., Reenstra W.W. Genistein potentiates wild-type and delta F508-CFTR channel activity. Am. J. Physiol. 273:1997;C988-C998.
-
(1997)
Am. J. Physiol.
, vol.273
-
-
Hwang, T.C.1
Wang, F.2
Yang, I.C.3
Reenstra, W.W.4
-
31
-
-
0028928015
-
CAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein
-
Illek B., Fisher H., Santos G.F., Widdicombe J.H., Machen T.E., Reenstra W.W. cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein. Am. J. Physiol. 268:1995;C886-C893.
-
(1995)
Am. J. Physiol.
, vol.268
-
-
Illek, B.1
Fisher, H.2
Santos, G.F.3
Widdicombe, J.H.4
Machen, T.E.5
Reenstra, W.W.6
-
32
-
-
0031763141
-
Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo
-
Illek B., Fisher H. Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo. Am. J. Physiol. 275:1998;L902-L910.
-
(1998)
Am. J. Physiol.
, vol.275
-
-
Illek, B.1
Fisher, H.2
-
33
-
-
0031028588
-
Modulation of CFTR chloride channels by calyculin A and genistein
-
Yang I.C., Cheng T.H., Wang F., Price E.M., Hwang T.C. Modulation of CFTR chloride channels by calyculin A and genistein. Am. J. Physiol. 272:1997;C142-C155.
-
(1997)
Am. J. Physiol.
, vol.272
-
-
Yang, I.C.1
Cheng, T.H.2
Wang, F.3
Price, E.M.4
Hwang, T.C.5
-
34
-
-
0026951303
-
Submucosal glands are the predominant site of CFTR expression in the human bronchus
-
Engelhardt J.F., Yankaskas J.R., Ernst S.A., Yang Y., Marino C.R., Boucher R.C., Cohn J.A., Wilson J.M. Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nat. Genet. 2:1992;240-248.
-
(1992)
Nat. Genet.
, vol.2
, pp. 240-248
-
-
Engelhardt, J.F.1
Yankaskas, J.R.2
Ernst, S.A.3
Yang, Y.4
Marino, C.R.5
Boucher, R.C.6
Cohn, J.A.7
Wilson, J.M.8
-
35
-
-
0033027269
-
Activation of wild-type and deltaF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms
-
Al-Nakkash L., Hwang T.C. Activation of wild-type and deltaF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms. Pflügers Arch. 437:1999;553-561.
-
(1999)
Pflügers Arch.
, vol.437
, pp. 553-561
-
-
Al-Nakkash, L.1
Hwang, T.C.2
|