-
1
-
-
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, Chinet T, Zierold K, Bonnet N, Puchelle E, and Balossier G. Abnormal ion content, hydration and granule expansion of the secretory granules from cystic fibrosis airway glandular cells. Exp Cell Res 309: 296-304, 2005.
-
(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
Chinet, T.11
Zierold, K.12
Bonnet, N.13
Puchelle, E.14
Balossier, G.15
-
3
-
-
0025784535
-
Defective acidification of intracellular organelles in cystic fibrosis
-
Barasch J, Kiss B, Prince A, Saiman L, Gruenert D, and al-Awqati Q. Defective acidification of intracellular organelles in cystic fibrosis. Nature 352: 70-73, 1991.
-
(1991)
Nature
, vol.352
, pp. 70-73
-
-
Barasch, J.1
Kiss, B.2
Prince, A.3
Saiman, L.4
Gruenert, D.5
Al-Awqati, Q.6
-
4
-
-
0026321416
-
Profound increase in viscosity and aggregation of pig gastric mucin at low pH
-
Bhaskar KR, Gong DH, Bansil R, Pajevic S, Hamilton JA, Turner BS, and LaMont JT. Profound increase in viscosity and aggregation of pig gastric mucin at low pH. Am J Physiol Gastrointest Liver Physiol 261: G827-G832, 1991.
-
(1991)
Am J Physiol Gastrointest Liver Physiol
, vol.261
-
-
Bhaskar, K.R.1
Gong, D.H.2
Bansil, R.3
Pajevic, S.4
Hamilton, J.A.5
Turner, B.S.6
LaMont, J.T.7
-
5
-
-
0031962056
-
Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-F508) cystic fibrosis transmembrane conductance regulator
-
Bhaskar KR, Turner BS, Grubman SA, Jefferson DM, and LaMont JT. Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-F508) cystic fibrosis transmembrane conductance regulator. Hepatology 27: 7-14, 1998.
-
(1998)
Hepatology
, vol.27
, pp. 7-14
-
-
Bhaskar, K.R.1
Turner, B.S.2
Grubman, S.A.3
Jefferson, D.M.4
LaMont, J.T.5
-
6
-
-
0026581842
-
Regulation of plasma membrane recycling by CFTR
-
Bradbury NA, Jilling T, Berta G, Sorscher EJ, Bridges RJ, and Kirk KL. Regulation of plasma membrane recycling by CFTR. Science 256: 530-532, 1992.
-
(1992)
Science
, vol.256
, pp. 530-532
-
-
Bradbury, N.A.1
Jilling, T.2
Berta, G.3
Sorscher, E.J.4
Bridges, R.J.5
Kirk, K.L.6
-
7
-
-
0035470157
-
Biosynthesis of mucin type O-glycans: Lack of correlation between glycosyltransferase and sulfotransferase activities and CFTR expression
-
Brockhausen I, Vavasseur F, and Yang X. Biosynthesis of mucin type O-glycans: lack of correlation between glycosyltransferase and sulfotransferase activities and CFTR expression. Glycoconj J 18: 685-697, 2001.
-
(2001)
Glycoconj J
, vol.18
, pp. 685-697
-
-
Brockhausen, I.1
Vavasseur, F.2
Yang, X.3
-
8
-
-
84985294642
-
Beam induced mass loss in high resolution biological microanalysis
-
Cantino ME, Wilkinson LE, Goddard MK, and Johnson DE. Beam induced mass loss in high resolution biological microanalysis. J Microsc 144: 317-327, 1986.
-
(1986)
J Microsc
, vol.144
, pp. 317-327
-
-
Cantino, M.E.1
Wilkinson, L.E.2
Goddard, M.K.3
Johnson, D.E.4
-
9
-
-
0024345843
-
Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis
-
Cheng PW, Boat TF, Cranfill K, Yankaskas JR, and Boucher RC. Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis. J Clin Invest 84: 68-72, 1989.
-
(1989)
J Clin Invest
, vol.84
, pp. 68-72
-
-
Cheng, P.W.1
Boat, T.F.2
Cranfill, K.3
Yankaskas, J.R.4
Boucher, R.C.5
-
10
-
-
0346103648
-
Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium
-
Coakley RD, Grubb BR, Paradiso AM, Gatzy JT, Johnson LG, Kreda SM, O'Neal WK, and Boucher RC. Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium. Proc Natl Acad Sci USA 100: 16083-16088, 2003.
-
(2003)
Proc Natl Acad Sci USA
, vol.100
, pp. 16083-16088
-
-
Coakley, R.D.1
Grubb, B.R.2
Paradiso, A.M.3
Gatzy, J.T.4
Johnson, L.G.5
Kreda, S.M.6
O'Neal, W.K.7
Boucher, R.C.8
-
11
-
-
0021365450
-
Counter-ion binding to mucus glycoproteins
-
Crowther RS and Marriott C. Counter-ion binding to mucus glycoproteins. J Pharm Pharmacol 36: 21-26, 1984.
-
(1984)
J Pharm Pharmacol
, vol.36
, pp. 21-26
-
-
Crowther, R.S.1
Marriott, C.2
-
12
-
-
30944452384
-
Mucus clearance and lung function in cystic fibrosis with hypertonic saline
-
Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, and Boucher RC. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 354: 241-250, 2006.
-
(2006)
N Engl J Med
, vol.354
, pp. 241-250
-
-
Donaldson, S.H.1
Bennett, W.D.2
Zeman, K.L.3
Knowles, M.R.4
Tarran, R.5
Boucher, R.C.6
-
13
-
-
30944466084
-
A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
-
Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB, Belousova EG, Xuan W, and Bye PT. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 354: 229-240, 2006.
-
(2006)
N Engl J Med
, vol.354
, pp. 229-240
-
-
Elkins, M.R.1
Robinson, M.2
Rose, B.R.3
Harbour, C.4
Moriarty, C.P.5
Marks, G.B.6
Belousova, E.G.7
Xuan, W.8
Bye, P.T.9
-
14
-
-
0028079278
-
Coupled secretion of chloride and mucus in skin of Xenopus laevis: Possible role for CFTR
-
Engelhardt JF, Smith SS, Allen E, Yankaskas JR, Dawson DC, and Wilson JM. Coupled secretion of chloride and mucus in skin of Xenopus laevis: possible role for CFTR. Am J Physiol Cell Physiol 267: C491-C500, 1994.
-
(1994)
Am J Physiol Cell Physiol
, vol.267
-
-
Engelhardt, J.F.1
Smith, S.S.2
Allen, E.3
Yankaskas, J.R.4
Dawson, D.C.5
Wilson, J.M.6
-
15
-
-
3042709453
-
MUC5AC and MUC5B mucins are decreased in cystic fibrosis airway secretions
-
Henke MO, Renner A, Huber RM, Seeds MC, and Rubin BK. MUC5AC and MUC5B mucins are decreased in cystic fibrosis airway secretions. Am J Respir Cell Mol Biol 31: 86-91, 2004.
-
(2004)
Am J Respir Cell Mol Biol
, vol.31
, pp. 86-91
-
-
Henke, M.O.1
Renner, A.2
Huber, R.M.3
Seeds, M.C.4
Rubin, B.K.5
-
16
-
-
0345269195
-
Novel Muc1 splice variants contribute to mucin overexpression in CFTR-deficient mice
-
Hinojosa-Kurtzberg AM, Johansson MEV, Madsen CS, Hansson GC, and Gendler SJ. Novel Muc1 splice variants contribute to mucin overexpression in CFTR-deficient mice. Am J Physiol Gastrointest Liver Physiol 284: G853-G862, 2003.
-
(2003)
Am J Physiol Gastrointest Liver Physiol
, vol.284
-
-
Hinojosa-Kurtzberg, A.M.1
Johansson, M.E.V.2
Madsen, C.S.3
Hansson, G.C.4
Gendler, S.J.5
-
17
-
-
0021994946
-
Electron microprobe analysis of human labial gland secretory granules in cystic fibrosis
-
Izutsu K, Johnson D, Schubert M, Wang E, Ramsey B, Tamarin A, Truelove E, Ensign W, and Young M. Electron microprobe analysis of human labial gland secretory granules in cystic fibrosis. J Clin Invest 75: 1951-1956, 1985.
-
(1985)
J Clin Invest
, vol.75
, pp. 1951-1956
-
-
Izutsu, K.1
Johnson, D.2
Schubert, M.3
Wang, E.4
Ramsey, B.5
Tamarin, A.6
Truelove, E.7
Ensign, W.8
Young, M.9
-
18
-
-
0030693691
-
Glycosylation differences between a cystic fibrosis and rescued airway cell line are not CFTR dependent
-
Jiang X, Hill WG, Pilewski JM, and Weisz OA. Glycosylation differences between a cystic fibrosis and rescued airway cell line are not CFTR dependent. Am J Physiol Lung Cell Mol Physiol 273: L913-L920, 1997.
-
(1997)
Am J Physiol Lung Cell Mol Physiol
, vol.273
-
-
Jiang, X.1
Hill, W.G.2
Pilewski, J.M.3
Weisz, O.A.4
-
19
-
-
0034972808
-
Characteristics of rodent intestinal mucin Muc3 and alterations in a mouse model of human cystic fibrosis
-
Khatri IA, Ho C, Specian RD, and Forstner JF. Characteristics of rodent intestinal mucin Muc3 and alterations in a mouse model of human cystic fibrosis. Am J Physiol Gastrointest Liver Physiol 280: G1321-G1330, 2001.
-
(2001)
Am J Physiol Gastrointest Liver Physiol
, vol.280
-
-
Khatri, I.A.1
Ho, C.2
Specian, R.D.3
Forstner, J.F.4
-
20
-
-
0028061411
-
Constitutive mucin secretion linked to CFTR expression
-
Kuver R, Ramesh N, Lau S, Savard C, Lee SP, and Osborne WR. Constitutive mucin secretion linked to CFTR expression. Biochem Biophys Res Commun 203: 1457-1462, 1994.
-
(1994)
Biochem Biophys Res Commun
, vol.203
, pp. 1457-1462
-
-
Kuver, R.1
Ramesh, N.2
Lau, S.3
Savard, C.4
Lee, S.P.5
Osborne, W.R.6
-
21
-
-
0030892737
-
Isolation and long-term culture of gallbladder epithelial cells from wild-type and CF mice
-
Kuver R, Savard C, Nguyen TD, Osborne WR, and Lee SP. Isolation and long-term culture of gallbladder epithelial cells from wild-type and CF mice. In Vitro Cell Dev Biol Anim 33: 104-109, 1997.
-
(1997)
In Vitro Cell Dev Biol Anim
, vol.33
, pp. 104-109
-
-
Kuver, R.1
Savard, C.2
Nguyen, T.D.3
Osborne, W.R.4
Lee, S.P.5
-
22
-
-
0034027661
-
Mucous granule exocytosis and CFTR expression in gallbladder epithelium
-
Kuver R, Klinkspoor JH, Osborne WR, and Lee SP. Mucous granule exocytosis and CFTR expression in gallbladder epithelium. Glycobiology 10: 149-157, 2000.
-
(2000)
Glycobiology
, vol.10
, pp. 149-157
-
-
Kuver, R.1
Klinkspoor, J.H.2
Osborne, W.R.3
Lee, S.P.4
-
23
-
-
0345743624
-
Calcium binding to biliary mucins is dependent on sodium ion concentration: Relevance to cystic fibrosis
-
Kuver R and Lee SP. Calcium binding to biliary mucins is dependent on sodium ion concentration: relevance to cystic fibrosis. Biochem Biophys Res Commun 314: 330-334, 2004.
-
(2004)
Biochem Biophys Res Commun
, vol.314
, pp. 330-334
-
-
Kuver, R.1
Lee, S.P.2
-
24
-
-
33646091498
-
Hypertonic saline for cystic fibrosis
-
(Letter)
-
Kuver R and Lee SP. Hypertonic saline for cystic fibrosis (Letter). N Engl J Med 354: 1848-1851, 2006.
-
(2006)
N Engl J Med
, vol.354
, pp. 1848-1851
-
-
Kuver, R.1
Lee, S.P.2
-
25
-
-
0018645420
-
Carbohydrate moieties of glycoproteins in human hepatic and gallbladder bile, gallbladder mucosa and gallstones
-
Lee SP, Lim TH, and Scott AJ. Carbohydrate moieties of glycoproteins in human hepatic and gallbladder bile, gallbladder mucosa and gallstones. Clin Sci (Lond) 56: 533-538, 1979.
-
(1979)
Clin Sci (Lond)
, vol.56
, pp. 533-538
-
-
Lee, S.P.1
Lim, T.H.2
Scott, A.J.3
-
26
-
-
18244390210
-
Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression
-
Leir SH, Parry S, Palmai-Pallag T, Evans J, Morris HR, Dell A, and Harris A. Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression. Am J Respir Cell Mol Biol 32: 453-461, 2005.
-
(2005)
Am J Respir Cell Mol Biol
, vol.32
, pp. 453-461
-
-
Leir, S.H.1
Parry, S.2
Palmai-Pallag, T.3
Evans, J.4
Morris, H.R.5
Dell, A.6
Harris, A.7
-
27
-
-
0031029855
-
Transcriptional activation of mucin by Pseudomonas aeruginosa lipopolysaccharide in the pathogenesis of cystic fibrosis lung disease
-
Li JD, Dohrman AF, Gallup M, Miyata S, Gum JR, Kim YS, Nadel JA, Prince A, and Basbaum CB. Transcriptional activation of mucin by Pseudomonas aeruginosa lipopolysaccharide in the pathogenesis of cystic fibrosis lung disease. Proc Natl Acad Sci USA 94: 967-972, 1997.
-
(1997)
Proc Natl Acad Sci USA
, vol.94
, pp. 967-972
-
-
Li, J.D.1
Dohrman, A.F.2
Gallup, M.3
Miyata, S.4
Gum, J.R.5
Kim, Y.S.6
Nadel, J.A.7
Prince, A.8
Basbaum, C.B.9
-
28
-
-
0017850731
-
A colorimetric assay for glycoproteins based on the periodic acid-Schiff stain
-
Mantle M and Allen A. A colorimetric assay for glycoproteins based on the periodic acid-Schiff stain. Biochem Soc Trans 6: 607-609, 1978.
-
(1978)
Biochem Soc Trans
, vol.6
, pp. 607-609
-
-
Mantle, M.1
Allen, A.2
-
29
-
-
0032533204
-
Cystic fibrosis mice lacking Muc1 have reduced amounts of intestinal mucus
-
Parmley RR and Gendler SJ. Cystic fibrosis mice lacking Muc1 have reduced amounts of intestinal mucus. J Clin Invest 102: 1798-1806, 1998.
-
(1998)
J Clin Invest
, vol.102
, pp. 1798-1806
-
-
Parmley, R.R.1
Gendler, S.J.2
-
30
-
-
5344221552
-
Reevaluating gel-forming mucins' roles in cystic fibrosis lung disease
-
Perez-Vilar J and Boucher RC. Reevaluating gel-forming mucins' roles in cystic fibrosis lung disease. Free Radic Biol Med 37: 1564-1577, 2004.
-
(2004)
Free Radic Biol Med
, vol.37
, pp. 1564-1577
-
-
Perez-Vilar, J.1
Boucher, R.C.2
-
31
-
-
33646169215
-
Mucin granule intraluminal organization in living mucous/goblet cells. Roles of protein post-translational modifications and secretion
-
Perez-Vilar J, Mabolo R, McVaugh CT, Bertozzi CR, and Boucher RC. Mucin granule intraluminal organization in living mucous/goblet cells. Roles of protein post-translational modifications and secretion. J Biol Chem 281: 4844-4855, 2006.
-
(2006)
J Biol Chem
, vol.281
, pp. 4844-4855
-
-
Perez-Vilar, J.1
Mabolo, R.2
McVaugh, C.T.3
Bertozzi, C.R.4
Boucher, R.C.5
-
32
-
-
0030473029
-
CFTR expression and mucin secretion in cultured mouse gallbladder epithelial cells
-
Peters RH, French PJ, van Doorninck JH, Lamblin G, Ratcliff R, Evans MJ, Colledge WH, Bijman J, and Scholte BJ. CFTR expression and mucin secretion in cultured mouse gallbladder epithelial cells. Am J Physiol Gastrointest Liver Physiol 271: G1074-G1083, 1996.
-
(1996)
Am J Physiol Gastrointest Liver Physiol
, vol.271
-
-
Peters, R.H.1
French, P.J.2
Van Doorninck, J.H.3
Lamblin, G.4
Ratcliff, R.5
Evans, M.J.6
Colledge, W.H.7
Bijman, J.8
Scholte, B.J.9
-
34
-
-
0016992401
-
Quantitative electron probe microanalysis of biological thin sections: Methods and validity
-
Shuman H, Somlyo AV, and Somlyo AP. Quantitative electron probe microanalysis of biological thin sections: methods and validity. Ultramicroscopy 1: 317-339, 1976.
-
(1976)
Ultramicroscopy
, vol.1
, pp. 317-339
-
-
Shuman, H.1
Somlyo, A.V.2
Somlyo, A.P.3
-
35
-
-
0020157812
-
Measurements of sulphate in body fluids
-
Silvestri LT, Hurst RE, Simpton L, and Settline JM. Measurements of sulphate in body fluids. Anal Biochem 123: 303-309, 1982.
-
(1982)
Anal Biochem
, vol.123
, pp. 303-309
-
-
Silvestri, L.T.1
Hurst, R.E.2
Simpton, L.3
Settline, J.M.4
-
36
-
-
0026649122
-
An animal model for cystic fibrosis made by gene targeting
-
Snouwaert JN, Brigman KK, Latour AM, Malouf NN, Boucher RC, Smithies O, and Koller BH. An animal model for cystic fibrosis made by gene targeting. Science 257: 1083-1088, 1992.
-
(1992)
Science
, vol.257
, pp. 1083-1088
-
-
Snouwaert, J.N.1
Brigman, K.K.2
Latour, A.M.3
Malouf, N.N.4
Boucher, R.C.5
Smithies, O.6
Koller, B.H.7
-
38
-
-
0016242360
-
Characterization of gastric mucoproteins isolated by equilibrium density-gradient centrifugation in caesium chloride
-
Starkey BJ, Snary D, and Allen A. Characterization of gastric mucoproteins isolated by equilibrium density-gradient centrifugation in caesium chloride. Biochem J 141: 633-639, 1974.
-
(1974)
Biochem J
, vol.141
, pp. 633-639
-
-
Starkey, B.J.1
Snary, D.2
Allen, A.3
-
39
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels
-
Stutts MJ, Canessa CM, Olsen JC, Hamrick M, Cohn JA, Rossier BC, and Boucher RC. CFTR as a cAMP-dependent regulator of sodium channels. Science 269: 847-850, 1995.
-
(1995)
Science
, vol.269
, pp. 847-850
-
-
Stutts, M.J.1
Canessa, C.M.2
Olsen, J.C.3
Hamrick, M.4
Cohn, J.A.5
Rossier, B.C.6
Boucher, R.C.7
-
40
-
-
0019426322
-
Control of mucus hydration as a Donnan equilibrium process
-
Tam PY and Verdugo P. Control of mucus hydration as a Donnan equilibrium process. Nature 292: 340-342, 1981.
-
(1981)
Nature
, vol.292
, pp. 340-342
-
-
Tam, P.Y.1
Verdugo, P.2
-
41
-
-
33748457432
-
Kinetics of swelling of gels
-
Tanaka T and Fillmore DJ. Kinetics of swelling of gels. J Chem Phys 70: 1214-1218, 1979.
-
(1979)
J Chem Phys
, vol.70
, pp. 1214-1218
-
-
Tanaka, T.1
Fillmore, D.J.2
-
42
-
-
0036847408
-
Intestinal mucins from cystic fibrosis mice show increased fucosylation due to an induced Fucα1-2 glycosyltransferase
-
Thomsson KA, Hinojosa-Kurtzberg M, Axelsson KA, Domino SE, Lowe JB, Gendler SJ, and Hansson GC. Intestinal mucins from cystic fibrosis mice show increased fucosylation due to an induced Fucα1-2 glycosyltransferase. Biochem J 367: 609-616, 2002.
-
(2002)
Biochem J
, vol.367
, pp. 609-616
-
-
Thomsson, K.A.1
Hinojosa-Kurtzberg, M.2
Axelsson, K.A.3
Domino, S.E.4
Lowe, J.B.5
Gendler, S.J.6
Hansson, G.C.7
-
44
-
-
0036712604
-
C-terminal domain of rodent intestinal mucin muc3 is proteolytically cleaved in the endoplasmic reticulum to generate extracellular and membrane components
-
Wang R, Khatri IA, and Forstner JF. C-terminal domain of rodent intestinal mucin muc3 is proteolytically cleaved in the endoplasmic reticulum to generate extracellular and membrane components. Biochem J 366: 623-631, 2002.
-
(2002)
Biochem J
, vol.366
, pp. 623-631
-
-
Wang, R.1
Khatri, I.A.2
Forstner, J.F.3
-
45
-
-
0028804840
-
Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis
-
Zhang Y, Doranz B, Yankaskas JR, and Engelhardt JF. Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis. J Clin Invest 96: 2997-3004, 1995.
-
(1995)
J Clin Invest
, vol.96
, pp. 2997-3004
-
-
Zhang, Y.1
Doranz, B.2
Yankaskas, J.R.3
Engelhardt, J.F.4
|