-
1
-
-
51349100398
-
The prevalence of cystic fibrosis in the European Union
-
Farrell, P. M. The prevalence of cystic fibrosis in the European Union. J. Cyst. Fibros. 7, 450-453 (2008).
-
(2008)
J. Cyst. Fibros.
, vol.7
, pp. 450-453
-
-
Farrell, P.M.1
-
2
-
-
85033702826
-
-
Cystic Fibrosis Foundation
-
Cystic Fibrosis Foundation. https://www.cff.org. 2017.
-
(2017)
-
-
-
3
-
-
85033717422
-
-
Cystic Fibrosis Canada
-
Cystic Fibrosis Canada. http://www.cysticfibrosis.ca/cf-care/cf-registry. 2017.
-
(2017)
-
-
-
4
-
-
85033706333
-
-
Cystic Fibrosis Australia
-
Cystic Fibrosis Australia. http://www.cysticfibrosis.org.au. 2017.
-
(2017)
-
-
-
6
-
-
36348979793
-
Sodium channels and cystic fibrosis
-
Donaldson, S. H. & Boucher, R. C. Sodium channels and cystic fibrosis. Chest 132, 1631-1636 (2007).
-
(2007)
Chest
, vol.132
, pp. 1631-1636
-
-
Donaldson, S.H.1
Boucher, R.C.2
-
7
-
-
84883445123
-
CFTR: A hub for kinases and crosstalk of cAMP and Ca2+
-
Kunzelmann, K. & Mehta, A. CFTR: a hub for kinases and crosstalk of cAMP and Ca2+. FEBS J. 280, 4417-4429 (2013).
-
(2013)
FEBS J.
, vol.280
, pp. 4417-4429
-
-
Kunzelmann, K.1
Mehta, A.2
-
8
-
-
84959908589
-
Acidic pH increases airway surface liquid viscosity in cystic fibrosis
-
Tang, X. X. et al. Acidic pH increases airway surface liquid viscosity in cystic fibrosis. J. Clin. Invest. 126, 879-891 (2016).
-
(2016)
J. Clin. Invest.
, vol.126
, pp. 879-891
-
-
Tang, X.X.1
-
9
-
-
0346103648
-
Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium
-
Coakley, R. D. et al. 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
-
10
-
-
0026645366
-
CAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia
-
Smith, J. J. & Welsh, M. J. cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia. J. Clin. Invest. 89, 1148-1153 (1992).
-
(1992)
J. Clin. Invest.
, vol.89
, pp. 1148-1153
-
-
Smith, J.J.1
Welsh, M.J.2
-
11
-
-
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. 279, C461-C479 (2000).
-
(2000)
Am. J. Physiol.
, vol.279
, pp. C461-C479
-
-
Devor, D.C.1
Bridges, R.J.2
Pilewski, J.M.3
-
12
-
-
84863476402
-
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
-
Pezzulo, A. A. et al. Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature 487, 109-113 (2012).
-
(2012)
Nature
, vol.487
, pp. 109-113
-
-
Pezzulo, A.A.1
-
13
-
-
84885084821
-
Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways
-
Garland, A. L. et al. Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways. Proc. Natl. Acad. Sci. USA 110, 15973-15978 (2013).
-
(2013)
Proc. Natl. Acad. Sci. USA
, vol.110
, pp. 15973-15978
-
-
Garland, A.L.1
-
14
-
-
84866103207
-
CFTR, mucins, and mucus obstruction in cystic fibrosis
-
Kreda, S. M., Davis, C. W. & Rose, M. C. CFTR, mucins, and mucus obstruction in cystic fibrosis. Cold Spring Harb. Perspect. Med. 2, a009589 (2012).
-
(2012)
Cold Spring Harb. Perspect. Med.
, vol.2
, pp. a009589
-
-
Kreda, S.M.1
Davis, C.W.2
Rose, M.C.3
-
15
-
-
0031885970
-
Influence of external pH on ciliary beat frequency in human bronchi and bronchioles
-
Clary-Meinesz, C., Mouroux, J., Cosson, J., Huitorel, P. & Blaive, B. Influence of external pH on ciliary beat frequency in human bronchi and bronchioles. Eur. Respir. J. 11, 330-333 (1998).
-
(1998)
Eur. Respir. J.
, vol.11
, pp. 330-333
-
-
Clary-Meinesz, C.1
Mouroux, J.2
Cosson, J.3
Huitorel, P.4
Blaive, B.5
-
16
-
-
0036137376
-
Acid stimulation reduces bactericidal activity of surface liquid in cultured human airway epithelial cells
-
Nakayama, K. et al. Acid stimulation reduces bactericidal activity of surface liquid in cultured human airway epithelial cells. Am. J. Respir. Cell Mol. Biol. 26, 105-113 (2002).
-
(2002)
Am. J. Respir. Cell Mol. Biol.
, vol.26
, pp. 105-113
-
-
Nakayama, K.1
-
17
-
-
0035048834
-
Acid exposure stimulates the adherence of Streptococcus pneumoniae to cultured human airway epithelial cells: Effects on plateletactivating factor receptor expression
-
Ishizuka, S. et al. Acid exposure stimulates the adherence of Streptococcus pneumoniae to cultured human airway epithelial cells: effects on plateletactivating factor receptor expression. Am. J. Respir. Cell Mol. Biol. 24, 459-468 (2001).
-
(2001)
Am. J. Respir. Cell Mol. Biol.
, vol.24
, pp. 459-468
-
-
Ishizuka, S.1
-
18
-
-
0033584935
-
Specific lipopolysaccharide found in cystic fibrosis airway Pseudomonas aeruginosa
-
Ernst, R. K. et al. Specific lipopolysaccharide found in cystic fibrosis airway Pseudomonas aeruginosa. Science 286, 1561-1565 (1999).
-
(1999)
Science
, vol.286
, pp. 1561-1565
-
-
Ernst, R.K.1
-
19
-
-
0020677346
-
Endobronchial pH Relevance of aminoglycoside activity in gram-negative bacillary pneumonia
-
Bodem, C. R., Lampton, L. M., Miller, D. P., Tarka, E. F. & Everett, E. D. Endobronchial pH. Relevance of aminoglycoside activity in gram-negative bacillary pneumonia. Am. Rev. Respir. Dis. 127, 39-41 (1983).
-
(1983)
Am. Rev. Respir. Dis.
, vol.127
, pp. 39-41
-
-
Bodem, C.R.1
Lampton, L.M.2
Miller, D.P.3
Tarka, E.F.4
Everett, E.D.5
-
20
-
-
84902333159
-
Neonates with cystic fibrosis have a reduced nasal liquid pH; A small pilot study
-
Abou Alaiwa, M. H. et al. Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study. J. Cyst. Fibros. 13, 373-377 (2014).
-
(2014)
J. Cyst. Fibros.
, vol.13
, pp. 373-377
-
-
Abou Alaiwa, M.H.1
-
21
-
-
0037252641
-
Airway surface pH in subjects with cystic fibrosis
-
McShane, D. et al. Airway surface pH in subjects with cystic fibrosis. Eur. Respir. J. 21, 37-42 (2003).
-
(2003)
Eur. Respir. J.
, vol.21
, pp. 37-42
-
-
McShane, D.1
-
22
-
-
84956707544
-
Airway acidification initiates host defense abnormalities in cystic fibrosis mice
-
Shah, V. S. et al. Airway acidification initiates host defense abnormalities in cystic fibrosis mice. Science 351, 503-507 (2016).
-
(2016)
Science
, vol.351
, pp. 503-507
-
-
Shah, V.S.1
-
23
-
-
25444449504
-
Predominant constitutive CFTR conductance in small airways
-
Wang, X., Lytle, C. & Quinton, P. M. Predominant constitutive CFTR conductance in small airways. Respir. Res. 6, 7 (2005).
-
(2005)
Respir. Res.
, vol.6
, pp. 7
-
-
Wang, X.1
Lytle, C.2
Quinton, P.M.3
-
24
-
-
22944457506
-
Materials for fluoresence-based optical chemical sensors
-
Wolfbeiz, O. S. Materials for fluoresence-based optical chemical sensors. J. Mater. Chem. 15, 2657-2669 (2005).
-
(2005)
J. Mater. Chem.
, vol.15
, pp. 2657-2669
-
-
Wolfbeiz, O.S.1
-
25
-
-
84891815327
-
Optical chemical pH sensors
-
Wencel, D., Abel, T. & McDonagh, C. Optical chemical pH sensors. Anal. Chem. 86, 15-29 (2014).
-
(2014)
Anal. Chem.
, vol.86
, pp. 15-29
-
-
Wencel, D.1
Abel, T.2
McDonagh, C.3
-
26
-
-
40449141631
-
Optical chemical sensors
-
McDonagh, C., Burke, C. S. & MacCraith, B. D. Optical chemical sensors. Chem. Rev. 108, 400-422 (2008).
-
(2008)
Chem. Rev.
, vol.108
, pp. 400-422
-
-
McDonagh, C.1
Burke, C.S.2
MacCraith, B.D.3
-
27
-
-
34848851105
-
Coordinated release of nucleotides and mucin from human airway epithelial Calu-3 cells
-
Kreda, S. M. et al. Coordinated release of nucleotides and mucin from human airway epithelial Calu-3 cells. J. Physiol. 584, 245-259 (2007).
-
(2007)
J. Physiol.
, vol.584
, pp. 245-259
-
-
Kreda, S.M.1
-
28
-
-
84992316555
-
Of pigs, mice, and men: Understanding early triggers of cystic fibrosis lung disease
-
Stick, S. M., Kicic, A. & Ranganathan, S. Of pigs, mice, and men: understanding early triggers of cystic fibrosis lung disease. Am. J. Respir. Crit. Care Med. 194, 784-785 (2016).
-
(2016)
Am. J. Respir. Crit. Care Med.
, vol.194
, pp. 784-785
-
-
Stick, S.M.1
Kicic, A.2
Ranganathan, S.3
-
29
-
-
0022995054
-
Development of the airway epithelium and submucosal glands in the pig lungs: Changes in epithelial glycoprotein profiles
-
Mills, A. M., Lopez-Vidriero, M. T. & Haworth, S. G. Development of the airway epithelium and submucosal glands in the pig lungs: changes in epithelial glycoprotein profiles. Br. J. Exp. Pathol. 67, 821-829 (1986).
-
(1986)
Br. J. Exp. Pathol.
, vol.67
, pp. 821-829
-
-
Mills, A.M.1
Lopez-Vidriero, M.T.2
Haworth, S.G.3
-
30
-
-
0036847496
-
Airways in cystic fibrosis are acidified: Detection by exhaled breath condensate
-
Tate, S., MacGregor, G., Davis, M., Innes, J. A. & Greening, A. P. Airways in cystic fibrosis are acidified: detection by exhaled breath condensate. Thorax 57, 926-929 (2002).
-
(2002)
Thorax
, vol.57
, pp. 926-929
-
-
Tate, S.1
MacGregor, G.2
Davis, M.3
Innes, J.A.4
Greening, A.P.5
-
31
-
-
84864590109
-
Highly photostable near-infrared fluorescent pH indicators and sensors based on BF2-chelated tetraarylazadipyrromethene dyes
-
Jokic, T. et al. Highly photostable near-infrared fluorescent pH indicators and sensors based on BF2-chelated tetraarylazadipyrromethene dyes. Anal. Chem. 84, 6723-6730 (2012).
-
(2012)
Anal. Chem.
, vol.84
, pp. 6723-6730
-
-
Jokic, T.1
-
32
-
-
84885015522
-
New life of ancient pigments: Application in high-performance optical sensing materials
-
Borisov, S. M., Würth, C., Resch-Genger, U. & Klimant, I. New life of ancient pigments: application in high-performance optical sensing materials. Anal. Chem. 85, 9371-9377 (2013).
-
(2013)
Anal. Chem.
, vol.85
, pp. 9371-9377
-
-
Borisov, S.M.1
Würth, C.2
Resch-Genger, U.3
Klimant, I.4
-
33
-
-
0000307701
-
-
Springer Series, (eds Valeur, B. & Brochon, J.-C Springer Berlin Heidelberg
-
Klimant, I. in New Trends in Fluorescence Spectroscopy. Springer Series, (eds Valeur, B. & Brochon, J.-C.) 1, 257-274 (Springer Berlin Heidelberg, 2001).
-
(2001)
New Trends in Fluorescence Spectroscopy
, vol.1
, pp. 257-274
-
-
Klimant, I.1
-
34
-
-
0035326478
-
Dual lifetime referencing as applied to a chloride optical sensor
-
Huber, C., Klimant, I., Krause, C. & Wolfbeis, O. S. Dual lifetime referencing as applied to a chloride optical sensor. Anal. Chem. 73, 2097-2103 (2001).
-
(2001)
Anal. Chem.
, vol.73
, pp. 2097-2103
-
-
Huber, C.1
Klimant, I.2
Krause, C.3
Wolfbeis, O.S.4
-
35
-
-
0025908613
-
Pharmacokinetic model driven infusion of propofol in children
-
Marsh, B., White, M., Morton, N. & Kenny, G. N. Pharmacokinetic model driven infusion of propofol in children. Br. J. Anaesth. 67, 41-48 (1991).
-
(1991)
Br. J. Anaesth.
, vol.67
, pp. 41-48
-
-
Marsh, B.1
White, M.2
Morton, N.3
Kenny, G.N.4
-
36
-
-
70349095833
-
Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis
-
Brennan, S. et al. Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis. Eur. Respir. J. 34, 655-661 (2009).
-
(2009)
Eur. Respir. J.
, vol.34
, pp. 655-661
-
-
Brennan, S.1
-
37
-
-
80052014087
-
Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis
-
Gangell, C. et al. Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis. Clin. Infect. Dis. 53, 425-432 (2011).
-
(2011)
Clin. Infect. Dis.
, vol.53
, pp. 425-432
-
-
Gangell, C.1
-
38
-
-
67650712233
-
Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening
-
Sly, P. D. et al. Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am. J. Respir. Crit. Care Med. 180, 146-152 (2009).
-
(2009)
Am. J. Respir. Crit. Care Med.
, vol.180
, pp. 146-152
-
-
Sly, P.D.1
-
39
-
-
80052528051
-
Primary epithelial cell models for cystic fibrosis research
-
Randell, S. H., Fulcher, M. L., O'Neal, W. & Olsen, J. C. Primary epithelial cell models for cystic fibrosis research. Methods Mol. Biol. 742, 285-310 (2011).
-
(2011)
Methods Mol. Biol.
, vol.742
, pp. 285-310
-
-
Randell, S.H.1
Fulcher, M.L.2
O'Neal, W.3
Olsen, J.C.4
-
40
-
-
16644381108
-
Well-differentiated human airway epithelial cell cultures
-
Fulcher, M. L., Gabriel, S., Burns, K. A., Yankaskas, J. R. & Randell, S. H. Well-differentiated human airway epithelial cell cultures. Methods Mol. Med. 107, 183-206 (2005).
-
(2005)
Methods Mol. Med.
, vol.107
, pp. 183-206
-
-
Fulcher, M.L.1
Gabriel, S.2
Burns, K.A.3
Yankaskas, J.R.4
Randell, S.H.5
-
41
-
-
66449127496
-
Endoplasmic reticulum/golgi nucleotide sugar transporters contribute to the cellular release of UDP-sugar signaling molecules
-
Sesma, J. I. et al. Endoplasmic reticulum/golgi nucleotide sugar transporters contribute to the cellular release of UDP-sugar signaling molecules. J. Biol. Chem. 284, 12572-12583 (2009).
-
(2009)
J. Biol. Chem.
, vol.284
, pp. 12572-12583
-
-
Sesma, J.I.1
-
42
-
-
84863042267
-
VAMP8 is a vesicle SNARE that regulates mucin secretion in airway goblet cells
-
Jones, L. C. et al. VAMP8 is a vesicle SNARE that regulates mucin secretion in airway goblet cells. J. Physiol. 590, 545-562 (2012).
-
(2012)
J. Physiol.
, vol.590
, pp. 545-562
-
-
Jones, L.C.1
|