-
1
-
-
77956651409
-
-
Karger, Switzerland, A. Bush, E.W.F.W. Alton, J.C. Davies, U. Griesenbach, A. Jaffe (Eds.)
-
st century, Basel 2006, 329. Karger, Switzerland. A. Bush, E.W.F.W. Alton, J.C. Davies, U. Griesenbach, A. Jaffe (Eds.).
-
(2006)
st century, Basel
, pp. 329
-
-
-
2
-
-
0036258208
-
Cystic fibrosis: a worldwide analysis of CFTR mutations-correlation with incidence data and application to screening
-
Bobadilla J.L., Jr M., Macek J.P.Fine, Farrell P.M. Cystic fibrosis: a worldwide analysis of CFTR mutations-correlation with incidence data and application to screening. Hum. Mutat. 2002, 19:575-606.
-
(2002)
Hum. Mutat.
, vol.19
, pp. 575-606
-
-
Bobadilla, J.L.1
Jr, M.2
Macek, J.P.F.3
Farrell, P.M.4
-
3
-
-
50649123290
-
CFTR function and prospects for therapy
-
Riordan J.R. CFTR function and prospects for therapy. Annu. Rev. Biochem. 2008, 77:701-726.
-
(2008)
Annu. Rev. Biochem.
, vol.77
, pp. 701-726
-
-
Riordan, J.R.1
-
4
-
-
77449160593
-
Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry
-
Lewis H.A., Wang C., Zhao X., Hamuro Y., Conners K., Kearins M.C., Lu F., Sauder J.M., Molnar K.S., Coales S.J., Maloney P.C., Guggino W.B., Wetmore D.R., Weber P.C., Hunt J.F. Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry. J. Mol. Biol. 2010, 396:406-430.
-
(2010)
J. Mol. Biol.
, vol.396
, pp. 406-430
-
-
Lewis, H.A.1
Wang, C.2
Zhao, X.3
Hamuro, Y.4
Conners, K.5
Kearins, M.C.6
Lu, F.7
Sauder, J.M.8
Molnar, K.S.9
Coales, S.J.10
Maloney, P.C.11
Guggino, W.B.12
Wetmore, D.R.13
Weber, P.C.14
Hunt, J.F.15
-
5
-
-
42149120706
-
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
-
Serohijos A.W., Hegedus T., Aleksandrov A.A., He L., Cui L., Dokholyan N.V., Riordan J.R. Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function. Proc. Natl Acad. Sci. USA 2008, 105:3256-3261.
-
(2008)
Proc. Natl Acad. Sci. USA
, vol.105
, pp. 3256-3261
-
-
Serohijos, A.W.1
Hegedus, T.2
Aleksandrov, A.A.3
He, L.4
Cui, L.5
Dokholyan, N.V.6
Riordan, J.R.7
-
6
-
-
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 1995, 83:121-127.
-
(1995)
Cell
, vol.83
, pp. 121-127
-
-
Ward, C.L.1
Omura, S.2
Kopito, R.R.3
-
7
-
-
33750842131
-
Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis
-
Wang X., Venable J., LaPointe P., Hutt D.M., Koulov A.V., Coppinger J., Gurkan C., Kellner W., Matteson J., Plutner H., Riordan J.R., Kelly J.W., Yates J.R., Balch W.E. Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 2006, 127:803-815.
-
(2006)
Cell
, vol.127
, pp. 803-815
-
-
Wang, X.1
Venable, J.2
LaPointe, P.3
Hutt, D.M.4
Koulov, A.V.5
Coppinger, J.6
Gurkan, C.7
Kellner, W.8
Matteson, J.9
Plutner, H.10
Riordan, J.R.11
Kelly, J.W.12
Yates, J.R.13
Balch, W.E.14
-
8
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning G.M., Anderson M.P., Amara J.F., Marshall J., Smith A.E., Welsh M.J. 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
-
9
-
-
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 R.G., Pavirani A., Lecocq J.P., 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
-
10
-
-
2542480784
-
Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator
-
Gentzsch M., Chang X.B., Cui L., Wu Y., Ozols V.V., Choudhury A., Pagano R.E., Riordan J.R. Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator. Mol. Biol. Cell 2004, 15:2684-2696.
-
(2004)
Mol. Biol. Cell
, vol.15
, pp. 2684-2696
-
-
Gentzsch, M.1
Chang, X.B.2
Cui, L.3
Wu, Y.4
Ozols, V.V.5
Choudhury, A.6
Pagano, R.E.7
Riordan, J.R.8
-
11
-
-
0027162649
-
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
-
Welsh M.J., Smith A.E. 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
-
12
-
-
0026907529
-
Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland
-
Kartner N., Augustinas O., Jensen T.J., Naismith A.L., Riordan J.R. Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland. Nat. Genet. 1992, 1:321-327.
-
(1992)
Nat. Genet.
, vol.1
, pp. 321-327
-
-
Kartner, N.1
Augustinas, O.2
Jensen, T.J.3
Naismith, A.L.4
Riordan, J.R.5
-
13
-
-
0032588980
-
DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis
-
Kälin N., Claass A., Sommer M., Puchelle E., Tümmler B. DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis. J. Clin. Invest. 1999, 103:1379-1389.
-
(1999)
J. Clin. Invest.
, vol.103
, pp. 1379-1389
-
-
Kälin, N.1
Claass, A.2
Sommer, M.3
Puchelle, E.4
Tümmler, B.5
-
14
-
-
0029082368
-
CFTR and differentiation markers expression in non-CF and delta F 508 homozygous CF nasal epithelium
-
Dupuit F., Kälin N., Brézillon S., Hinnrasky J., Tümmler B., Puchelle E. CFTR and differentiation markers expression in non-CF and delta F 508 homozygous CF nasal epithelium. J. Clin. Invest. 1995, 96:1601-1611.
-
(1995)
J. Clin. Invest.
, vol.96
, pp. 1601-1611
-
-
Dupuit, F.1
Kälin, N.2
Brézillon, S.3
Hinnrasky, J.4
Tümmler, B.5
Puchelle, E.6
-
15
-
-
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., Malhó R., Amaral M.D. Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells. Lab. Invest. 2000, 80:857-868.
-
(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
Malhó, R.8
Amaral, M.D.9
-
16
-
-
0036840749
-
Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation
-
Sermet-Gaudelus I., Vallée B., Urbin I., Torossi T., Marianovski R., Fajac A., Feuillet M.N., Bresson J.L., Lenoir G., Bernaudin J.F., Edelman A. Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation. Pediatr. Res. 2002, 52:628-635.
-
(2002)
Pediatr. Res.
, vol.52
, pp. 628-635
-
-
Sermet-Gaudelus, I.1
Vallée, B.2
Urbin, I.3
Torossi, T.4
Marianovski, R.5
Fajac, A.6
Feuillet, M.N.7
Bresson, J.L.8
Lenoir, G.9
Bernaudin, J.F.10
Edelman, A.11
-
17
-
-
18244377969
-
Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia
-
Kreda S.M., Mall M., Mengos A., Rochelle L., Yankaskas J., Riordan J.R., Boucher R.C. 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
-
18
-
-
0035213684
-
Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings
-
Bronsveld I., Mekus F., Bijman J., Ballmann M., de Jonge H.R., Laabs U., Halley D.J., Ellemunter H., Mastella G., Thomas S., Veeze H.J., Tümmler B. Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings. J. Clin. Invest. 2001, 108:1705-1715.
-
(2001)
J. Clin. Invest.
, vol.108
, pp. 1705-1715
-
-
Bronsveld, I.1
Mekus, F.2
Bijman, J.3
Ballmann, M.4
de Jonge, H.R.5
Laabs, U.6
Halley, D.J.7
Ellemunter, H.8
Mastella, G.9
Thomas, S.10
Veeze, H.J.11
Tümmler, B.12
-
19
-
-
0347361558
-
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon
-
Mall M., Kreda S.M., Mengos A., Jensen T.J., Hirtz S., Seydewitz H.H., Yankaskas J., Kunzelmann K., Riordan J.R., Boucher R.C. The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon. Gastroenterology 2004, 126:32-41.
-
(2004)
Gastroenterology
, vol.126
, pp. 32-41
-
-
Mall, M.1
Kreda, S.M.2
Mengos, A.3
Jensen, T.J.4
Hirtz, S.5
Seydewitz, H.H.6
Yankaskas, J.7
Kunzelmann, K.8
Riordan, J.R.9
Boucher, R.C.10
-
20
-
-
34347333381
-
Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis
-
Amaral M.D., Kunzelmann K. Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis. Trends Pharmacol. Sci. 2007, 28:334-341.
-
(2007)
Trends Pharmacol. Sci.
, vol.28
, pp. 334-341
-
-
Amaral, M.D.1
Kunzelmann, K.2
-
21
-
-
0034234755
-
Residual chloride secretion in intestinal tissue of deltaF508 homozygous twins and siblings with cystic fibrosis
-
Bronsveld I., Mekus F., Bijman J., Ballmann M., Greipel J., Hundrieser J., Halley D.J., Laabs U., Busche R., De Jonge H.R., Tümmler B., Veeze H.J. Residual chloride secretion in intestinal tissue of deltaF508 homozygous twins and siblings with cystic fibrosis. Gastroenterology 2000, 119:32-40.
-
(2000)
Gastroenterology
, vol.119
, pp. 32-40
-
-
Bronsveld, I.1
Mekus, F.2
Bijman, J.3
Ballmann, M.4
Greipel, J.5
Hundrieser, J.6
Halley, D.J.7
Laabs, U.8
Busche, R.9
De Jonge, H.R.10
Tümmler, B.11
Veeze, H.J.12
-
22
-
-
33644746288
-
Ex vivo biochemical analysis of CFTR in human rectal biopsies
-
van Barneveld A., Stanke F., Ballmann M., Naim N.Y., Tümmler B. Ex vivo biochemical analysis of CFTR in human rectal biopsies. Biochim. Biophys. Acta 2006, 1762:393-397.
-
(2006)
Biochim. Biophys. Acta
, vol.1762
, pp. 393-397
-
-
van Barneveld, A.1
Stanke, F.2
Ballmann, M.3
Naim, N.Y.4
Tümmler, B.5
-
23
-
-
0017184389
-
A rapid and sensitive for the quantitation of microgram quantitites of protein utilizing the principle of protein-dye binding
-
Bradford M.M. A rapid and sensitive for the quantitation of microgram quantitites of protein utilizing the principle of protein-dye binding. Anal. Biochem. 1976, 72:248-254.
-
(1976)
Anal. Biochem.
, vol.72
, pp. 248-254
-
-
Bradford, M.M.1
-
24
-
-
0023088808
-
Biosynthesis and maturation of lactase-phlorizin hydrolase in the human small intestinal epithelial cells
-
Naim H.Y., Sterchi E.E., Lentze M.J. Biosynthesis and maturation of lactase-phlorizin hydrolase in the human small intestinal epithelial cells. Biochem. J. 1987, 241:427-434.
-
(1987)
Biochem. J.
, vol.241
, pp. 427-434
-
-
Naim, H.Y.1
Sterchi, E.E.2
Lentze, M.J.3
-
25
-
-
0023783489
-
Sucrase-isomaltase deficiency in humans. Different mutations disrupt intracellular transport, processing, and function of an intestinal brush border enzyme
-
Naim H.Y., Roth J., Sterchi E.E., Lentze M., Milla P., Schmitz J., Hauri H.P. Sucrase-isomaltase deficiency in humans. Different mutations disrupt intracellular transport, processing, and function of an intestinal brush border enzyme. J. Clin. Invest. 1988, 82:667-679.
-
(1988)
J. Clin. Invest.
, vol.82
, pp. 667-679
-
-
Naim, H.Y.1
Roth, J.2
Sterchi, E.E.3
Lentze, M.4
Milla, P.5
Schmitz, J.6
Hauri, H.P.7
-
26
-
-
38349101872
-
Diversity of the basic defect of homozygous CFTR mutation genotypes in humans
-
Stanke F., Ballmann M., Bronsveld I., Dörk T., Gallati S., Laabs U., Derichs N., Ritzka M., Posselt H.G., Harms H.K., Griese M., Blau H., Mastella G., Bijman J., Veeze H., Tümmler B. Diversity of the basic defect of homozygous CFTR mutation genotypes in humans. J. Med. Genet. 2008, 45:47-54.
-
(2008)
J. Med. Genet.
, vol.45
, pp. 47-54
-
-
Stanke, F.1
Ballmann, M.2
Bronsveld, I.3
Dörk, T.4
Gallati, S.5
Laabs, U.6
Derichs, N.7
Ritzka, M.8
Posselt, H.G.9
Harms, H.K.10
Griese, M.11
Blau, H.12
Mastella, G.13
Bijman, J.14
Veeze, H.15
Tümmler, B.16
-
27
-
-
77955298789
-
Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data
-
Derichs N., Sanz J., von Kanel T., Stolpe C., Zapf A., Tümmler B., Gallati S., Ballmann M. Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data. Thorax 2010, 65:594-599.
-
(2010)
Thorax
, vol.65
, pp. 594-599
-
-
Derichs, N.1
Sanz, J.2
von Kanel, T.3
Stolpe, C.4
Zapf, A.5
Tümmler, B.6
Gallati, S.7
Ballmann, M.8
-
28
-
-
11044232030
-
Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers
-
De Jonge H.R., Ballmann M., Veeze H., Bronsveld I., Stanke F., Tümmler B., Sinaasappel M. Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers. J. Cyst. Fibros. 2004, 3(Suppl. 2):159-163.
-
(2004)
J. Cyst. Fibros.
, vol.3
, Issue.SUPPL. 2
, pp. 159-163
-
-
De Jonge, H.R.1
Ballmann, M.2
Veeze, H.3
Bronsveld, I.4
Stanke, F.5
Tümmler, B.6
Sinaasappel, M.7
-
29
-
-
0025772974
-
Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis
-
Veeze H.J., Sinaasappel M., Bijman J., Bouquet J., de Jonge H.R. Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis. Gastroenterology 1991, 101:398-403.
-
(1991)
Gastroenterology
, vol.101
, pp. 398-403
-
-
Veeze, H.J.1
Sinaasappel, M.2
Bijman, J.3
Bouquet, J.4
de Jonge, H.R.5
-
30
-
-
0028218460
-
Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype
-
Veeze H.J., Halley D.J., Bijman J., de Jongste J.C., de Jonge H.R., Sinaasappel M. Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype. J. Clin. Invest. 1994, 93:461-466.
-
(1994)
J. Clin. Invest.
, vol.93
, pp. 461-466
-
-
Veeze, H.J.1
Halley, D.J.2
Bijman, J.3
de Jongste, J.C.4
de Jonge, H.R.5
Sinaasappel, M.6
-
31
-
-
38349033879
-
Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator
-
Schmidt A., Hughes L.K., Cai Z., Mendes F., Li H., Sheppard D.N., Amaral M.D. Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator. Br. J. Pharmacol. 2008, 153:1311-1323.
-
(2008)
Br. J. Pharmacol.
, vol.153
, pp. 1311-1323
-
-
Schmidt, A.1
Hughes, L.K.2
Cai, Z.3
Mendes, F.4
Li, H.5
Sheppard, D.N.6
Amaral, M.D.7
-
32
-
-
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. 1995, 269:G874-882.
-
(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
-
33
-
-
0032415114
-
Cholinergic ion secretion in human colon requires coactivation by cAMP
-
Mall M., Bleich M., Schürlein M., Kühr J., Seydewitz H.H., Brandis M., Greger R., Kunzelmann K. Cholinergic ion secretion in human colon requires coactivation by cAMP. Am. J. Physiol. 1998, 275:G1274-1281.
-
(1998)
Am. J. Physiol.
, vol.275
-
-
Mall, M.1
Bleich, M.2
Schürlein, M.3
Kühr, J.4
Seydewitz, H.H.5
Brandis, M.6
Greger, R.7
Kunzelmann, K.8
-
34
-
-
73249114731
-
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
-
Van Goor F., Hadida S., Grootenhuis P.D., Burton B., Cao D., Neuberger T., Turnbull A., Singh A., Joubran J., Hazlewood A., Zhou J., McCartney J., Arumugam V., Decker C., Yang J., Young C., Olson E.R., Wine J.J., Frizzell R.A., Ashlock M., Negulescu P. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc. Natl Acad. Sci. USA 2009, 106:18825-18830.
-
(2009)
Proc. Natl Acad. Sci. USA
, vol.106
, pp. 18825-18830
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
Burton, B.4
Cao, D.5
Neuberger, T.6
Turnbull, A.7
Singh, A.8
Joubran, J.9
Hazlewood, A.10
Zhou, J.11
McCartney, J.12
Arumugam, V.13
Decker, C.14
Yang, J.15
Young, C.16
Olson, E.R.17
Wine, J.J.18
Frizzell, R.A.19
Ashlock, M.20
Negulescu, P.21
more..
-
35
-
-
77951807825
-
Bestrophin-2 mediates bicarbonate transport by goblet cells in mouse colon
-
Yu K., Lujan R., Marmorstein A., Gabriel S., Hartzell H.C. Bestrophin-2 mediates bicarbonate transport by goblet cells in mouse colon. J. Clin. Invest. 2010, 120:1722-1735.
-
(2010)
J. Clin. Invest.
, vol.120
, pp. 1722-1735
-
-
Yu, K.1
Lujan, R.2
Marmorstein, A.3
Gabriel, S.4
Hartzell, H.C.5
-
36
-
-
9444288754
-
The CLCA gene locus as a modulator of the gastrointestinal basic defect in cystic fibrosis
-
Ritzka M., Stanke F., Jansen S., Gruber A.D., Pusch L., Woelfl S., Veeze H.J., Halley D.J., Tümmler B. The CLCA gene locus as a modulator of the gastrointestinal basic defect in cystic fibrosis. Hum. Genet. 2004, 115:483-491.
-
(2004)
Hum. Genet.
, vol.115
, pp. 483-491
-
-
Ritzka, M.1
Stanke, F.2
Jansen, S.3
Gruber, A.D.4
Pusch, L.5
Woelfl, S.6
Veeze, H.J.7
Halley, D.J.8
Tümmler, B.9
-
37
-
-
73549089880
-
Epac1 mediates protein kinase A-independent mechanism of forskolin-activated intestinal chloride secretion
-
Hoque K.M., Woodward O.M., van Rossum D.B., Zachos N.C., Chen L., Leung G.P., Guggino W.B., Guggino S.E., Tse C.M. Epac1 mediates protein kinase A-independent mechanism of forskolin-activated intestinal chloride secretion. J. Gen. Physiol. 2010, 135:43-58.
-
(2010)
J. Gen. Physiol.
, vol.135
, pp. 43-58
-
-
Hoque, K.M.1
Woodward, O.M.2
van Rossum, D.B.3
Zachos, N.C.4
Chen, L.5
Leung, G.P.6
Guggino, W.B.7
Guggino, S.E.8
Tse, C.M.9
-
38
-
-
33745772850
-
New insights into cystic fibrosis: molecular switches that regulate CFTR
-
Guggino W.B., Stanton B.A. New insights into cystic fibrosis: molecular switches that regulate CFTR. Nat. Rev. Mol. Cell Biol. 2006, 7:426-436.
-
(2006)
Nat. Rev. Mol. Cell Biol.
, vol.7
, pp. 426-436
-
-
Guggino, W.B.1
Stanton, B.A.2
-
39
-
-
33645311204
-
Lipid raft organization and function in brush borders of epithelial cells
-
Danielsen E.M., Hansen G.H. Lipid raft organization and function in brush borders of epithelial cells. Mol. Membr. Biol. 2006, 23:71-79.
-
(2006)
Mol. Membr. Biol.
, vol.23
, pp. 71-79
-
-
Danielsen, E.M.1
Hansen, G.H.2
-
40
-
-
0036479131
-
A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression
-
Cheng J., Moyer B.D., Milewski M., Loffing J., Ikeda M., Mickle J.E., Cutting G.R., Li M., Stanton B.A., Guggino W.B. A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression. J. Biol. Chem. 2002, 277:3520-3529.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 3520-3529
-
-
Cheng, J.1
Moyer, B.D.2
Milewski, M.3
Loffing, J.4
Ikeda, M.5
Mickle, J.E.6
Cutting, G.R.7
Li, M.8
Stanton, B.A.9
Guggino, W.B.10
-
41
-
-
41849092177
-
Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis
-
Teichgräber V., Ulrich M., Endlich N., Riethmüller J., Wilker B., De Oliveira-Munding C.C., van Heeckeren A.M., Barr M.L., von Kürthy G., Schmid K.W., Weller M., Tümmler B., Lang F., Grassme H., Döring G., Gulbins E. Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat. Med. 2008, 14:382-391.
-
(2008)
Nat. Med.
, vol.14
, pp. 382-391
-
-
Teichgräber, V.1
Ulrich, M.2
Endlich, N.3
Riethmüller, J.4
Wilker, B.5
De Oliveira-Munding, C.C.6
van Heeckeren, A.M.7
Barr, M.L.8
von Kürthy, G.9
Schmid, K.W.10
Weller, M.11
Tümmler, B.12
Lang, F.13
Grassme, H.14
Döring, G.15
Gulbins, E.16
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