-
1
-
-
46749117136
-
Molecular and cellular aspects of protein misfolding and disease
-
Herczenik E, Gebbink MF. Molecular and cellular aspects of protein misfolding and disease. FASEB J 2008;22:2115-2133.
-
(2008)
FASEB J
, vol.22
, pp. 2115-2133
-
-
Herczenik, E.1
Gebbink, M.F.2
-
3
-
-
46149097167
-
What's new in cystic fibrosis? From treating symptoms to correction of the basic defect
-
Proesmans M, Vermeulen F, De Boeck K. What's new in cystic fibrosis? From treating symptoms to correction of the basic defect. Eur J Pediatr 2008;167:839-849.
-
(2008)
Eur J Pediatr
, vol.167
, pp. 839-849
-
-
Proesmans, M.1
Vermeulen, F.2
De Boeck, K.3
-
4
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE, Welsh MJ. 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
Marshall, J.4
Smith, A.E.5
Welsh, M.J.6
-
5
-
-
0026325533
-
Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation
-
Dalemans W, Barbry P, Champigny G, Jallat S, Dott K, Dreyer D, Crystal RG, Pavirani A, Lecocq JP, Lazdunski M. Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation. Nature 1991;354:526-528.
-
(1991)
Nature
, vol.354
, pp. 526-528
-
-
Dalemans, W.1
Barbry, P.2
Champigny, G.3
Jallat, S.4
Dott, K.5
Dreyer, D.6
Crystal, R.G.7
Pavirani, A.8
Lecocq, J.P.9
Lazdunski, M.10
-
6
-
-
0030042386
-
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
-
Sato S, Ward CL, Krouse ME, Wine JJ, Kopito RR. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J Biol Chem 1996;271:635-638.
-
(1996)
J Biol Chem
, vol.271
, pp. 635-638
-
-
Sato, S.1
Ward, C.L.2
Krouse, M.E.3
Wine, J.J.4
Kopito, R.R.5
-
7
-
-
0036115199
-
Calcium-pump inhibitors induce functional surface expression of delta F508-CFTR protein in cystic fibrosis epithelial cells
-
Egan ME, Glockner-Pagel J, Ambrose C, Cahill PA, Pappoe L, Balamuth N, Cho E, Canny S,Wagner CA,Geibel J,etal.Calcium-pump inhibitors induce functional surface expression of delta F508-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.3
Cahill, P.A.4
Pappoe, L.5
Balamuth, N.6
Cho, E.7
Canny, S.8
Wagner, C.A.9
Geibel, J.10
-
8
-
-
11144355340
-
Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects
-
Egan ME, Pearson M, Weiner SA, Rajendran V, Rubin D, Glockner- Pagel J, Canny S, Du K, Lukacs GL, Caplan MJ. 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
Rajendran, V.4
Rubin, D.5
Glockner- Pagel, J.6
Canny, S.7
Du, K.8
Lukacs, G.L.9
Caplan, M.J.10
-
9
-
-
0034804969
-
S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation
-
Zaman K, McPherson M, Vaughan J, Hunt J, Mendes F, Gaston B, Palmer LA. S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation. Biochem Biophys Res Commun 2001;284:65-70.
-
(2001)
Biochem Biophys Res Commun
, vol.284
, pp. 65-70
-
-
Zaman, K.1
McPherson, M.2
Vaughan, J.3
Hunt, J.4
Mendes, F.5
Gaston, B.6
Palmer, L.A.7
-
10
-
-
33748944330
-
S-nitrosylating agents: A novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells
-
Zaman K, Carraro S, Doherty J, Henderson EM, Lendermon E, Liu L, Verghese G, Zigler M, Ross M, Park E, et al. S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells. Mol Pharmacol 2006;70:1435-1442.
-
(2006)
Mol Pharmacol
, vol.70
, pp. 1435-1442
-
-
Zaman, K.1
Carraro, S.2
Doherty, J.3
Henderson, E.M.4
Lendermon, E.5
Liu, L.6
Verghese, G.7
Zigler, M.8
Ross, M.9
Park, E.10
-
11
-
-
0034099743
-
Sodium 4-phenylbutryate downregulates HSC70: Implications for intracellular trafficking of ΔF508-CFTR
-
Rubenstein RC, Zeitlin PL. Sodium 4-phenylbutryate downregulates HSC70: implications for intracellular trafficking of ΔF508-CFTR. Am J Physiol 2000;278:C259-C267.
-
(2000)
Am J Physiol
, vol.278
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
12
-
-
24644464284
-
Small-molecule correctors of defective deltaF508-CFTR cellular processing identified by high-throughput screening
-
Pedemonte N, Lukacs GL, Du K, Caci E, Zegarra-Moran O, Galietta LJ, Verkman AS. Small-molecule correctors of defective deltaF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest 2005;115:2564-2571.
-
(2005)
J Clin Invest
, vol.115
, pp. 2564-2571
-
-
Pedemonte, N.1
Lukacs, G.L.2
Du, K.3
Caci, E.4
Zegarra-Moran, O.5
Galietta, L.J.6
Verkman, A.S.7
-
13
-
-
33744831154
-
Rescue of deltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
-
Van Goor F, Straley KS, Cao D, Gonzalez J, Hadida S, Hazlewood A, Joubran J, Knapp T, Makings LR, Miller M, et al. Rescue of deltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol 2006;290:L1117-L1130.
-
(2006)
Am J Physiol
, vol.290
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
Gonzalez, J.4
Hadida, S.5
Hazlewood, A.6
Joubran, J.7
Knapp, T.8
Makings, L.R.9
Miller, M.10
-
14
-
-
34547557698
-
Correctors of protein trafficking defects identified by a novel high-throughput screening assay
-
Carlile GW, Robert R, Zhang D, Teske KA, Luo Y, Hanrahan JW, Thomas DY. Correctors of protein trafficking defects identified by a novel high-throughput screening assay. ChemBioChem 2007;8:1012- 1020.
-
(2007)
ChemBioChem
, vol.8
, pp. 1012-1020
-
-
Carlile, G.W.1
Robert, R.2
Zhang, D.3
Teske, K.A.4
Luo, Y.5
Hanrahan, J.W.6
Thomas, D.Y.7
-
15
-
-
38549125726
-
Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect
-
Robert R, Carlile GW, Pavel C, Liu N, Anjos SM, Liao J, Luo Y, Zhang D, Thomas DY, Hanrahan JW. Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect. Mol Pharmacol 2008;73:478-489.
-
(2008)
Mol Pharmacol
, vol.73
, pp. 478-489
-
-
Robert, R.1
Carlile, G.W.2
Pavel, C.3
Liu, N.4
Anjos, S.M.5
Liao, J.6
Luo, Y.7
Zhang, D.8
Thomas, D.Y.9
Hanrahan, J.W.10
-
16
-
-
63349111698
-
Salmeterol restores secretory functions in cystic fibrosis airway submucosal gland serous cells
-
Delavoie F, Molinari M, Milliot M, Zahm J-M, Coraux C, Michel J, Balossier G. Salmeterol restores secretory functions in cystic fibrosis airway submucosal gland serous cells. Am J Respir Cell Mol Biol 2009; 40:388-397.
-
(2009)
Am J Respir Cell Mol Biol
, vol.40
, pp. 388-397
-
-
Delavoie, F.1
Molinari, M.2
Milliot, M.3
Zahm, J.-M.4
Coraux, C.5
Michel, J.6
Balossier, G.7
-
17
-
-
0026951303
-
Submucosal glands are the predominant site of CFTR expression in the human bronchus
-
Engelhardt JF, Yankaskas JR, Ernst SA, Yang Y, Marino CR, Boucher RC, Cohn JA, Wilson JM. Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nat Genet 1992;2:240-248.
-
(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
-
18
-
-
18244377969
-
Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia
-
Kreda SM, Mall M, Mengos A, Rochelle L, Yankaskas J, Riordan JR, Boucher RC. Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia. Mol Biol Cell 2005;16:2154-2167.
-
(2005)
Mol Biol Cell
, vol.16
, pp. 2154-2167
-
-
Kreda, S.M.1
Mall, M.2
Mengos, A.3
Rochelle, L.4
Yankaskas, J.5
Riordan, J.R.6
Boucher, R.C.7
-
19
-
-
33846245581
-
Acinar origin of CFTR-dependent airway submucosal gland fluid secretion
-
Wu JV, Krouse ME, Wine JJ. Acinar origin of CFTR-dependent airway submucosal gland fluid secretion. Am J Physiol 2007;292:L304-L311.
-
(2007)
Am J Physiol
, vol.292
-
-
Wu, J.V.1
Krouse, M.E.2
Wine, J.J.3
-
20
-
-
0027131093
-
Receptors that couple to 2 classes of G proteins increase camp and activate CFTR expressed in xenopus oocytes
-
Uezono Y, Bradley J, Min C, McCarty NA, Quick M, Riordan JR, Chavkin C, Zinn K, Lester HA, Davidson N. Receptors that couple to 2 classes of G proteins increase camp and activate CFTR expressed in xenopus oocytes. Receptors Channels 1993;1:233-241.
-
(1993)
Receptors Channels
, vol.1
, pp. 233-241
-
-
Uezono, Y.1
Bradley, J.2
Min, C.3
McCarty, N.A.4
Quick, M.5
Riordan, J.R.6
Chavkin, C.7
Zinn, K.8
Lester, H.A.9
Davidson, N.10
-
21
-
-
0037422547
-
A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phos-phoprotein 50 is regulated by PKA
-
Naren AP, Cobb B, Li C, Roy K, Nelson D, Heda GD, Liao J, Kirk KL, Sorscher EJ, Hanrahan J, et al. A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phos-phoprotein 50 is regulated by PKA. Proc Natl Acad Sci USA 2003; 100:342-346.
-
(2003)
Proc Natl Acad Sci USA
, vol.100
, pp. 342-346
-
-
Naren, A.P.1
Cobb, B.2
Li, C.3
Roy, K.4
Nelson, D.5
Heda, G.D.6
Liao, J.7
Kirk, K.L.8
Sorscher, E.J.9
Hanrahan, J.10
-
22
-
-
0023025504
-
Cystic fibrosis and beta-adrenergic response of airway epithelial cell cultures
-
Widdicombe JH. Cystic fibrosis and beta-adrenergic response of airway epithelial cell cultures. Am J Physiol 1986;251:R818-R822.
-
(1986)
Am J Physiol
, vol.251
-
-
Widdicombe, J.H.1
-
24
-
-
0037930844
-
Stimulation of beta 2-adrenergic receptor increases cystic fibrosis transmembrane conductance regulator expression in human airway epithelial cells through a cAMP/protein kinase a-independent pathway
-
Taouil K, Hinnrasky J, Hologne C, Corlieu P, Klossek JM, Puchelle E. Stimulation of beta 2-adrenergic receptor increases cystic fibrosis transmembrane conductance regulator expression in human airway epithelial cells through a cAMP/protein kinase a-independent pathway. J Biol Chem 2003;278:17320-17327.
-
(2003)
J Biol Chem
, vol.278
, pp. 17320-17327
-
-
Taouil, K.1
Hinnrasky, J.2
Hologne, C.3
Corlieu, P.4
Klossek, J.M.5
Puchelle, E.6
-
25
-
-
0344390884
-
A PDZ-interacting domain in CFTR is an apical membrane polarization signal
-
Moyer BD, Denton J, Karlson KH, Reynolds D, Wang S, Mickle JE, Milewski M, Cutting GR, Guggino WB, Li M, et al. A PDZ-interacting domain in CFTR is an apical membrane polarization signal. J Clin Invest 1999;104:1353-1361.
-
(1999)
J Clin Invest
, vol.104
, pp. 1353-1361
-
-
Moyer, B.D.1
Denton, J.2
Karlson, K.H.3
Reynolds, D.4
Wang, S.5
Mickle, J.E.6
Milewski, M.7
Cutting, G.R.8
Guggino, W.B.9
Li, M.10
-
26
-
-
0032555156
-
-
+ exchanger regulator factor family of PDZ proteins. Proc Natl Acad Sci USA 1998;95:8496-8501.
-
+ exchanger regulator factor family of PDZ proteins. Proc Natl Acad Sci USA 1998;95:8496-8501.
-
-
-
-
27
-
-
0032498963
-
+ exchange
-
+ exchange. Nature 1998;392:626-630.
-
(1998)
Nature
, vol.392
, pp. 626-630
-
-
Hall, R.A.1
Premont, R.T.2
Chow, C.W.3
Blitzer, J.T.4
Pitcher, J.A.5
Claing, A.6
Stoffel, R.H.7
Barak, L.S.8
Shenolikars, S.9
Weinman, E.J.10
-
29
-
-
0037131297
-
PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator
-
Swiatecka-Urban A, Duhaime M, Coutermarsh B, Karlson KH, Collawn J, Milewski M, Cutting GR, Guggino WB, Langford G, Stanton BA. PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator. J Biol Chem 2002; 277:40099-40105.
-
(2002)
J Biol Chem
, vol.277
, pp. 40099-40105
-
-
Swiatecka-Urban, A.1
Duhaime, M.2
Coutermarsh, B.3
Karlson, K.H.4
Collawn, J.5
Milewski, M.6
Cutting, G.R.7
Guggino, W.B.8
Langford, G.9
Stanton, B.A.10
-
30
-
-
24944557160
-
Abnormal ion content, hydration and granule expansion of the secretory granules from cystic fibrosis airway glandular cells
-
Baconnais S, Delavoie F, Zahm JM, Milliot M, Terryn C, Castillon N, Banchet V, Michel J, Danos O, Merten M, et al. Abnormal ion content, hydration and granule expansion of the secretory granules from cystic fibrosis airway glandular cells. Exp Cell Res 2005;309:296-304.
-
(2005)
Exp Cell Res
, vol.309
, pp. 296-304
-
-
Baconnais, S.1
Delavoie, F.2
Zahm, J.M.3
Milliot, M.4
Terryn, C.5
Castillon, N.6
Banchet, V.7
Michel, J.8
Danos, O.9
Merten, M.10
-
31
-
-
0029841642
-
Sustained activation of a G protein-coupled receptor via ''anchored'' agonist binding: Molecular localization of the salmeterol exosite within the 2-adrenergic receptor
-
Green SA, Spasoff AP, Coleman RA, Johnson M, Liggett SB. Sustained activation of a G protein-coupled receptor via ''anchored'' agonist binding: molecular localization of the salmeterol exosite within the 2-adrenergic receptor. J Biol Chem 1996;271:24029-24035.
-
(1996)
J Biol Chem
, vol.271
, pp. 24029-24035
-
-
Green, S.A.1
Spasoff, A.P.2
Coleman, R.A.3
Johnson, M.4
Liggett, S.B.5
-
32
-
-
0031990692
-
Salmeterol-induced desensitization, internal-ization and phosphorylation of the human beta2-adrenoceptor
-
January B, Seibold A, Allal C, Whaley BS, Knoll BJ, Moore RH, Dickey BF, Barber R, Clark RB. Salmeterol-induced desensitization, internal-ization and phosphorylation of the human beta2-adrenoceptor. Br J Pharmacol 1998;123:701-711.
-
(1998)
Br J Pharmacol
, vol.123
, pp. 701-711
-
-
January, B.1
Seibold, A.2
Allal, C.3
Whaley, B.S.4
Knoll, B.J.5
Moore, R.H.6
Dickey, B.F.7
Barber, R.8
Clark, R.B.9
-
33
-
-
33846895899
-
Salmeterol stimulation dissociates beta2-adrenergic receptor phosphorylation and internalization
-
Moore RH, Millman EE, Godines V, Hanania NA, Tran TM, Peng H, Dickey BF, Knoll BJ, Clark RB. Salmeterol stimulation dissociates beta2-adrenergic receptor phosphorylation and internalization. Am J Respir Cell Mol Biol 2007;36:254-261.
-
(2007)
Am J Respir Cell Mol Biol
, vol.36
, pp. 254-261
-
-
Moore, R.H.1
Millman, E.E.2
Godines, V.3
Hanania, N.A.4
Tran, T.M.5
Peng, H.6
Dickey, B.F.7
Knoll, B.J.8
Clark, R.B.9
-
34
-
-
0028236014
-
Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel
-
Prince LS, Workman RB Jr, Marchase RB. Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel. Proc Natl Acad Sci USA 1994;91:5192-5196.
-
(1994)
Proc Natl Acad Sci USA
, vol.91
, pp. 5192-5196
-
-
Prince, L.S.1
Workman Jr, R.B.2
Marchase, R.B.3
-
35
-
-
34547646140
-
Respiratory syncytial virus induces insensitivity to b-adrenergic agonists in mouse lung epithelium in vivo
-
Davis IC, Xu A, Gao Z, Hickman-Davis JM, Factor P, Sullender WM, Matalon S. Respiratory syncytial virus induces insensitivity to b-adrenergic agonists in mouse lung epithelium in vivo. Am J Physiol 2007;293:L281-L289.
-
(2007)
Am J Physiol
, vol.293
-
-
Davis, I.C.1
Xu, A.2
Gao, Z.3
Hickman-Davis, J.M.4
Factor, P.5
Sullender, W.M.6
Matalon, S.7
|