-
1
-
-
0036194724
-
Mucus clearance as a primary innate defense mechanism for mammalian airways
-
Knowles M.R., Boucher R.C. Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest 2002, 109(5):571-577.
-
(2002)
J Clin Invest
, vol.109
, Issue.5
, pp. 571-577
-
-
Knowles, M.R.1
Boucher, R.C.2
-
2
-
-
1042269503
-
Innate immunity in the lung: how epithelial cells fight against respiratory pathogens
-
Bals R., Hiemstra P.S. Innate immunity in the lung: how epithelial cells fight against respiratory pathogens. Eur Respir J 2004, 23(2):327-333.
-
(2004)
Eur Respir J
, vol.23
, Issue.2
, pp. 327-333
-
-
Bals, R.1
Hiemstra, P.S.2
-
3
-
-
67349161534
-
Barrier functions and paracellular integrity in human cell culture models of the proximal respiratory unit
-
Pohl C., Hermanns M.I., Uboldi C., et al. Barrier functions and paracellular integrity in human cell culture models of the proximal respiratory unit. Eur J Pharm Biopharm 2009, 72(2):339-349.
-
(2009)
Eur J Pharm Biopharm
, vol.72
, Issue.2
, pp. 339-349
-
-
Pohl, C.1
Hermanns, M.I.2
Uboldi, C.3
-
4
-
-
84879285251
-
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 2012, 2(9):a009589.
-
(2012)
Cold Spring Harb Perspect Med
, vol.2
, Issue.9
, pp. a009589
-
-
Kreda, S.M.1
Davis, C.W.2
Rose, M.C.3
-
5
-
-
84906089250
-
Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis
-
Hoegger M.J., Fischer A.J., McMenimen J.D., et al. Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis. Science 2014, 345(6198):818-822.
-
(2014)
Science
, vol.345
, Issue.6198
, pp. 818-822
-
-
Hoegger, M.J.1
Fischer, A.J.2
McMenimen, J.D.3
-
6
-
-
78649647597
-
Role of epithelial HCO3(-) transport in mucin secretion: lessons from cystic fibrosis
-
Quinton P.M. Role of epithelial HCO3(-) transport in mucin secretion: lessons from cystic fibrosis. Am J Physiol Cell Physiol 2010, 299(6):C1222-C1233.
-
(2010)
Am J Physiol Cell Physiol
, vol.299
, Issue.6
, pp. C1222-C1233
-
-
Quinton, P.M.1
-
7
-
-
84906707282
-
Microbial-induced meprin beta cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus
-
Schutte A., Ermund A., Becker-Pauly C., et al. Microbial-induced meprin beta cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus. Proc Natl Acad Sci U S A 2014, 111(34):12396-12401.
-
(2014)
Proc Natl Acad Sci U S A
, vol.111
, Issue.34
, pp. 12396-12401
-
-
Schutte, A.1
Ermund, A.2
Becker-Pauly, C.3
-
8
-
-
34047166052
-
Airway surface dehydration in cystic fibrosis: pathogenesis and therapy
-
Boucher R.C. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med 2007, 58:157-170.
-
(2007)
Annu Rev Med
, vol.58
, pp. 157-170
-
-
Boucher, R.C.1
-
9
-
-
4344619319
-
Nucleotide release provides a mechanism for airway surface liquid homeostasis
-
Lazarowski E.R., Tarran R., Grubb B.R., et al. Nucleotide release provides a mechanism for airway surface liquid homeostasis. J Biol Chem 2004, 279(35):36855-36864.
-
(2004)
J Biol Chem
, vol.279
, Issue.35
, pp. 36855-36864
-
-
Lazarowski, E.R.1
Tarran, R.2
Grubb, B.R.3
-
10
-
-
84923870560
-
Oxidation increases mucin polymer cross-links to stiffen airway mucus gels
-
276ra27.
-
Yuan S., Hollinger M., Lachowicz-Scroggins M.E., et al. Oxidation increases mucin polymer cross-links to stiffen airway mucus gels. Sci Transl Med 2015, 7(276):276ra27.
-
(2015)
Sci Transl Med
, vol.7
, Issue.276
-
-
Yuan, S.1
Hollinger, M.2
Lachowicz-Scroggins, M.E.3
-
11
-
-
0036167851
-
Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients
-
Worlitzsch D., Tarran R., Ulrich M., et al. Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients. J Clin Invest 2002, 109(3):317-325.
-
(2002)
J Clin Invest
, vol.109
, Issue.3
, pp. 317-325
-
-
Worlitzsch, D.1
Tarran, R.2
Ulrich, M.3
-
12
-
-
84908073532
-
Gene expression in whole lung and pulmonary macrophages reflects the dynamic pathology associated with airway surface dehydration
-
Saini Y., Dang H., Livraghi-Butrico A., et al. Gene expression in whole lung and pulmonary macrophages reflects the dynamic pathology associated with airway surface dehydration. BMC Genomics 2014, 15:726.
-
(2014)
BMC Genomics
, vol.15
, pp. 726
-
-
Saini, Y.1
Dang, H.2
Livraghi-Butrico, A.3
-
13
-
-
84924565068
-
PH modulates the activity and synergism of the airway surface liquid antimicrobials beta-defensin-3 and LL-37
-
Abou Alaiwa M.H., Reznikov L.R., Gansemer N.D., et al. pH modulates the activity and synergism of the airway surface liquid antimicrobials beta-defensin-3 and LL-37. Proc Natl Acad Sci U S A 2014, 111(52):18703-18708.
-
(2014)
Proc Natl Acad Sci U S A
, vol.111
, Issue.52
, pp. 18703-18708
-
-
Abou Alaiwa, M.H.1
Reznikov, L.R.2
Gansemer, N.D.3
-
14
-
-
4644263748
-
Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis
-
Rogan M.P., Taggart C.C., Greene C.M., et al. Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis. J Infect Dis 2004, 190(7):1245-1253.
-
(2004)
J Infect Dis
, vol.190
, Issue.7
, pp. 1245-1253
-
-
Rogan, M.P.1
Taggart, C.C.2
Greene, C.M.3
-
15
-
-
84878370385
-
Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients
-
Ghio A.J., Roggli V.L., Soukup J.M., et al. Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients. J Cyst Fibros 2013, 12(4):390-398.
-
(2013)
J Cyst Fibros
, vol.12
, Issue.4
, pp. 390-398
-
-
Ghio, A.J.1
Roggli, V.L.2
Soukup, J.M.3
-
16
-
-
84928315351
-
Combination of hypothiocyanite and lactoferrin (ALX-109) enhances the ability of tobramycin and aztreonam to eliminate Pseudomonas aeruginosa biofilms growing on cystic fibrosis airway epithelial cells
-
Moreau-Marquis S., Coutermarsh B., Stanton B.A. Combination of hypothiocyanite and lactoferrin (ALX-109) enhances the ability of tobramycin and aztreonam to eliminate Pseudomonas aeruginosa biofilms growing on cystic fibrosis airway epithelial cells. J Antimicrob Chemother 2015, 70(1):160-166.
-
(2015)
J Antimicrob Chemother
, vol.70
, Issue.1
, pp. 160-166
-
-
Moreau-Marquis, S.1
Coutermarsh, B.2
Stanton, B.A.3
-
17
-
-
84905992903
-
A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy
-
Pohl K., Hayes E., Keenan J., et al. A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy. Blood 2014, 124(7):999-1009.
-
(2014)
Blood
, vol.124
, Issue.7
, pp. 999-1009
-
-
Pohl, K.1
Hayes, E.2
Keenan, J.3
-
18
-
-
84929468871
-
Bactericidal/Permeability-increasing protein fold-containing family member A1 in airway host protection and respiratory disease
-
Britto C.J., Cohn L. Bactericidal/Permeability-increasing protein fold-containing family member A1 in airway host protection and respiratory disease. Am J Respir Cell Mol Biol 2015, 52(5):525-534.
-
(2015)
Am J Respir Cell Mol Biol
, vol.52
, Issue.5
, pp. 525-534
-
-
Britto, C.J.1
Cohn, L.2
-
19
-
-
84901608363
-
Mammalian short palate lung and nasal epithelial clone 1 (SPLUNC1) in pH-dependent airway hydration
-
Tarran R., Redinbo M.R. Mammalian short palate lung and nasal epithelial clone 1 (SPLUNC1) in pH-dependent airway hydration. Int J Biochem Cell Biol 2014, 52:130-135.
-
(2014)
Int J Biochem Cell Biol
, vol.52
, pp. 130-135
-
-
Tarran, R.1
Redinbo, M.R.2
-
20
-
-
0022485439
-
Lysosomal-enzymes from polymorphonuclear leukocytes and proteinase-inhibitors in patients with cystic-fibrosis
-
Goldstein W., Doring G. Lysosomal-enzymes from polymorphonuclear leukocytes and proteinase-inhibitors in patients with cystic-fibrosis. Am Rev Respir Dis 1986, 134(1):49-56.
-
(1986)
Am Rev Respir Dis
, vol.134
, Issue.1
, pp. 49-56
-
-
Goldstein, W.1
Doring, G.2
-
21
-
-
84868296268
-
Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis
-
Sagel S.D., Wagner B.D., Anthony M.M., et al. Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis. Am J Respir Crit Care Med 2012, 186(9):857-865.
-
(2012)
Am J Respir Crit Care Med
, vol.186
, Issue.9
, pp. 857-865
-
-
Sagel, S.D.1
Wagner, B.D.2
Anthony, M.M.3
-
22
-
-
0021783739
-
Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibrosis
-
Bruce M.C., Poncz L., Klinger J.D., et al. Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibrosis. Am Rev Respir Dis 1985, 132(3):529-535.
-
(1985)
Am Rev Respir Dis
, vol.132
, Issue.3
, pp. 529-535
-
-
Bruce, M.C.1
Poncz, L.2
Klinger, J.D.3
-
23
-
-
36849020586
-
Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease
-
Hartl D., Latzin P., Hordijk P., et al. Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease. Nat Med 2007, 13(12):1423-1430.
-
(2007)
Nat Med
, vol.13
, Issue.12
, pp. 1423-1430
-
-
Hartl, D.1
Latzin, P.2
Hordijk, P.3
-
24
-
-
0036195343
-
Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis
-
Vandivier R.W., Fadok V.A., Hoffmann P.R., et al. Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis. J Clin Invest 2002, 109(5):661-670.
-
(2002)
J Clin Invest
, vol.109
, Issue.5
, pp. 661-670
-
-
Vandivier, R.W.1
Fadok, V.A.2
Hoffmann, P.R.3
-
25
-
-
0027998135
-
The role of neutrophil elastase in chronic inflammation
-
Doring G. The role of neutrophil elastase in chronic inflammation. Am J Respir Crit Care Med 1994, 150(6 Pt 2):S114-S117.
-
(1994)
Am J Respir Crit Care Med
, vol.150
, Issue.6
, pp. S114-S117
-
-
Doring, G.1
-
26
-
-
0033045546
-
Neutrophil elastase increases MUC5AC mRNA and protein expression in respiratory epithelial cells
-
Voynow J.A., Young L.R., Wang Y., et al. Neutrophil elastase increases MUC5AC mRNA and protein expression in respiratory epithelial cells. Am J Physiol 1999, 276(5 Pt 1):L835-L843.
-
(1999)
Am J Physiol
, vol.276
, Issue.5
, pp. L835-L843
-
-
Voynow, J.A.1
Young, L.R.2
Wang, Y.3
-
27
-
-
0038018539
-
Neutrophil elastase up-regulates interleukin-8 via toll-like receptor 4
-
Devaney J.M., Greene C.M., Taggart C.C., et al. Neutrophil elastase up-regulates interleukin-8 via toll-like receptor 4. FEBS Lett 2003, 544(1-3):129-132.
-
(2003)
FEBS Lett
, vol.544
, Issue.1-3
, pp. 129-132
-
-
Devaney, J.M.1
Greene, C.M.2
Taggart, C.C.3
-
28
-
-
84906952377
-
Network GIotCFFTD. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis
-
Rowe S.M., Heltshe S.L., Gonska T., et al. Network GIotCFFTD. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Am J Respir Crit Care Med 2014, 190(2):175-184.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, Issue.2
, pp. 175-184
-
-
Rowe, S.M.1
Heltshe, S.L.2
Gonska, T.3
-
29
-
-
84906922269
-
MiR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production
-
Weldon S., McNally P., McAuley D.F., et al. miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production. Am J Respir Crit Care Med 2014, 190(2):165-174.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, Issue.2
, pp. 165-174
-
-
Weldon, S.1
McNally, P.2
McAuley, D.F.3
-
30
-
-
0033113290
-
Proteinase 3, a potent secretagogue in airways, is present in cystic fibrosis sputum
-
Witko-Sarsat V., Halbwachs-Mecarelli L., Schuster A., et al. Proteinase 3, a potent secretagogue in airways, is present in cystic fibrosis sputum. Am J Respir Cell Mol Biol 1999, 20(4):729-736.
-
(1999)
Am J Respir Cell Mol Biol
, vol.20
, Issue.4
, pp. 729-736
-
-
Witko-Sarsat, V.1
Halbwachs-Mecarelli, L.2
Schuster, A.3
-
31
-
-
80053063761
-
The role of matrix metalloproteinases in cystic fibrosis lung disease
-
Gaggar A., Hector A., Bratcher P.E., et al. The role of matrix metalloproteinases in cystic fibrosis lung disease. Eur Respir J 2011, 38(3):721-727.
-
(2011)
Eur Respir J
, vol.38
, Issue.3
, pp. 721-727
-
-
Gaggar, A.1
Hector, A.2
Bratcher, P.E.3
-
32
-
-
84858294319
-
Oxidative stress and antioxidant therapy in cystic fibrosis
-
Galli F., Battistoni A., Gambari R., et al. Oxidative stress and antioxidant therapy in cystic fibrosis. Biochim Biophys Acta 2012, 1822(5):690-713.
-
(2012)
Biochim Biophys Acta
, vol.1822
, Issue.5
, pp. 690-713
-
-
Galli, F.1
Battistoni, A.2
Gambari, R.3
-
33
-
-
0034769118
-
Nitric oxide and the immune response
-
Bogdan C. Nitric oxide and the immune response. Nat Immunol 2001, 2(10):907-916.
-
(2001)
Nat Immunol
, vol.2
, Issue.10
, pp. 907-916
-
-
Bogdan, C.1
-
34
-
-
0030961127
-
Decreased concentration of exhaled nitric oxide (NO) in patients with cystic fibrosis
-
Grasemann H., Michler E., Wallot M., et al. Decreased concentration of exhaled nitric oxide (NO) in patients with cystic fibrosis. Pediatr Pulmonol 1997, 24(3):173-177.
-
(1997)
Pediatr Pulmonol
, vol.24
, Issue.3
, pp. 173-177
-
-
Grasemann, H.1
Michler, E.2
Wallot, M.3
-
35
-
-
0032531160
-
Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells
-
Kelley T.J., Drumm M.L. Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells. J Clin Invest 1998, 102(6):1200-1207.
-
(1998)
J Clin Invest
, vol.102
, Issue.6
, pp. 1200-1207
-
-
Kelley, T.J.1
Drumm, M.L.2
-
36
-
-
30444439420
-
Increased arginase activity in cystic fibrosis airways
-
Grasemann H., Schwiertz R., Matthiesen S., et al. Increased arginase activity in cystic fibrosis airways. Am J Respir Crit Care Med 2005, 172(12):1523-1528.
-
(2005)
Am J Respir Crit Care Med
, vol.172
, Issue.12
, pp. 1523-1528
-
-
Grasemann, H.1
Schwiertz, R.2
Matthiesen, S.3
-
37
-
-
84946490523
-
Effect of ivacaftor therapy on exhaled nitric oxide in patients with cystic fibrosis
-
Grasemann H., Gonska T., Avolio J., et al. Effect of ivacaftor therapy on exhaled nitric oxide in patients with cystic fibrosis. J Cyst Fibros 2015, 14(6):727-732.
-
(2015)
J Cyst Fibros
, vol.14
, Issue.6
, pp. 727-732
-
-
Grasemann, H.1
Gonska, T.2
Avolio, J.3
-
38
-
-
84946488323
-
Concentration of fractional excretion of nitric oxide (FENO): a potential airway biomarker of restored CFTR function
-
Kotha K., Szczesniak R.D., Naren A.P., et al. Concentration of fractional excretion of nitric oxide (FENO): a potential airway biomarker of restored CFTR function. J Cyst Fibros 2015, 14(6):733-740.
-
(2015)
J Cyst Fibros
, vol.14
, Issue.6
, pp. 733-740
-
-
Kotha, K.1
Szczesniak, R.D.2
Naren, A.P.3
-
39
-
-
0036735754
-
Regulation of airway tight junctions by proinflammatory cytokines
-
Coyne C.B., Vanhook M.K., Gambling T.M., et al. Regulation of airway tight junctions by proinflammatory cytokines. Mol Biol Cell 2002, 13(9):3218-3234.
-
(2002)
Mol Biol Cell
, vol.13
, Issue.9
, pp. 3218-3234
-
-
Coyne, C.B.1
Vanhook, M.K.2
Gambling, T.M.3
-
40
-
-
0347126488
-
Src signaling links mediators of inflammation to Cx43 gap junction channels in primary and transformed CFTR-expressing airway cells
-
Huang S., Jornot L., Wiszniewski L., et al. Src signaling links mediators of inflammation to Cx43 gap junction channels in primary and transformed CFTR-expressing airway cells. Cell Commun Adhes 2003, 10(4-6):279-285.
-
(2003)
Cell Commun Adhes
, vol.10
, Issue.4-6
, pp. 279-285
-
-
Huang, S.1
Jornot, L.2
Wiszniewski, L.3
-
41
-
-
84940766346
-
Junctional abnormalities in human airway epithelial cells expressing F508del CFTR
-
Molina S.A., Stauffer B., Moriarty H.K., et al. Junctional abnormalities in human airway epithelial cells expressing F508del CFTR. Am J Physiol Lung Cell Mol Physiol 2015, 309(5):L475-L487.
-
(2015)
Am J Physiol Lung Cell Mol Physiol
, vol.309
, Issue.5
, pp. L475-L487
-
-
Molina, S.A.1
Stauffer, B.2
Moriarty, H.K.3
-
42
-
-
80052098128
-
Paracellular permeability of bronchial epithelium is controlled by CFTR
-
Weiser N., Molenda N., Urbanova K., et al. Paracellular permeability of bronchial epithelium is controlled by CFTR. Cell Physiol Biochem 2011, 28(2):289-296.
-
(2011)
Cell Physiol Biochem
, vol.28
, Issue.2
, pp. 289-296
-
-
Weiser, N.1
Molenda, N.2
Urbanova, K.3
-
43
-
-
84893657016
-
Disrupted tight junctions in the small intestine of cystic fibrosis mice
-
De Lisle R.C. Disrupted tight junctions in the small intestine of cystic fibrosis mice. Cell Tissue Res 2014, 355(1):131-142.
-
(2014)
Cell Tissue Res
, vol.355
, Issue.1
, pp. 131-142
-
-
De Lisle, R.C.1
-
44
-
-
84937736412
-
Airway epithelial cell integrity protects from cytotoxicity of Pseudomonas aeruginosa quorum-sensing signals
-
Losa D., Kohler T., Bacchetta M., et al. Airway epithelial cell integrity protects from cytotoxicity of Pseudomonas aeruginosa quorum-sensing signals. Am J Respir Cell Mol Biol 2015, 53(2):265-275.
-
(2015)
Am J Respir Cell Mol Biol
, vol.53
, Issue.2
, pp. 265-275
-
-
Losa, D.1
Kohler, T.2
Bacchetta, M.3
-
45
-
-
41949134634
-
CFTR in a lipid raft-TNFR1 complex modulates gap junctional intercellular communication and IL-8 secretion
-
Dudez T., Borot F., Huang S., et al. CFTR in a lipid raft-TNFR1 complex modulates gap junctional intercellular communication and IL-8 secretion. Biochim Biophys Acta 2008, 1783(5):779-788.
-
(2008)
Biochim Biophys Acta
, vol.1783
, Issue.5
, pp. 779-788
-
-
Dudez, T.1
Borot, F.2
Huang, S.3
-
46
-
-
79959572825
-
Abnormal trafficking and degradation of TLR4 underlie the elevated inflammatory response in cystic fibrosis
-
Bruscia E.M., Zhang P.-X., Satoh A., et al. Abnormal trafficking and degradation of TLR4 underlie the elevated inflammatory response in cystic fibrosis. J Immunol 2011, 186(12):6990-6998.
-
(2011)
J Immunol
, vol.186
, Issue.12
, pp. 6990-6998
-
-
Bruscia, E.M.1
Zhang, P.-X.2
Satoh, A.3
-
47
-
-
20444362700
-
Gap junctional communication in tissue inflammation and repair
-
Chanson M., Derouette J.P., Roth I., et al. Gap junctional communication in tissue inflammation and repair. Biochim Biophys Acta 2005, 1711(2):197-207.
-
(2005)
Biochim Biophys Acta
, vol.1711
, Issue.2
, pp. 197-207
-
-
Chanson, M.1
Derouette, J.P.2
Roth, I.3
-
48
-
-
33646463903
-
Novel effects of azithromycin on tight junction proteins in human airway epithelia
-
Asgrimsson V., Gudjonsson T., Gudmundsson G.H., et al. Novel effects of azithromycin on tight junction proteins in human airway epithelia. Antimicrob Agents Chemother 2006, 50(5):1805-1812.
-
(2006)
Antimicrob Agents Chemother
, vol.50
, Issue.5
, pp. 1805-1812
-
-
Asgrimsson, V.1
Gudjonsson, T.2
Gudmundsson, G.H.3
-
49
-
-
32944464648
-
Pathogen recognition and innate immunity
-
Akira S., Uematsu S., Takeuchi O. Pathogen recognition and innate immunity. Cell 2006, 124(4):783-801.
-
(2006)
Cell
, vol.124
, Issue.4
, pp. 783-801
-
-
Akira, S.1
Uematsu, S.2
Takeuchi, O.3
-
50
-
-
0033844485
-
Inflammation and infection in naive human cystic fibrosis airway grafts
-
Tirouvanziam R., de Bentzmann S., Hubeau C., et al. Inflammation and infection in naive human cystic fibrosis airway grafts. Am J Respir Cell Mol Biol 2000, 23(2):121-127.
-
(2000)
Am J Respir Cell Mol Biol
, vol.23
, Issue.2
, pp. 121-127
-
-
Tirouvanziam, R.1
de Bentzmann, S.2
Hubeau, C.3
-
51
-
-
0034774914
-
Distinct pattern of immune cell population in the lung of human fetuses with cystic fibrosis
-
Hubeau C., Puchelle E., Gaillard D. Distinct pattern of immune cell population in the lung of human fetuses with cystic fibrosis. J Allergy Clin Immunol 2001, 108(4):524-529.
-
(2001)
J Allergy Clin Immunol
, vol.108
, Issue.4
, pp. 524-529
-
-
Hubeau, C.1
Puchelle, E.2
Gaillard, D.3
-
52
-
-
0028784322
-
Inflammatory cytokines in cystic fibrosis lungs
-
Bonfield T.L., Panuska J.R., Konstan M.W., et al. Inflammatory cytokines in cystic fibrosis lungs. Am J Respir Crit Care Med 1995, 152(6 Pt 1):2111-2118.
-
(1995)
Am J Respir Crit Care Med
, vol.152
, Issue.6
, pp. 2111-2118
-
-
Bonfield, T.L.1
Panuska, J.R.2
Konstan, M.W.3
-
53
-
-
77953428335
-
Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): potential mediators of cystic fibrosis airway inflammation
-
Gaggar A., Rowe S.M., Matthew H., et al. Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): potential mediators of cystic fibrosis airway inflammation. Open Respir Med J 2010, 4:32-38.
-
(2010)
Open Respir Med J
, vol.4
, pp. 32-38
-
-
Gaggar, A.1
Rowe, S.M.2
Matthew, H.3
-
54
-
-
54049139979
-
Potential role of high-mobility group box 1 in cystic fibrosis airway disease
-
Rowe S.M., Jackson P.L., Liu G., et al. Potential role of high-mobility group box 1 in cystic fibrosis airway disease. Am J Respir Crit Care Med 2008, 178(8):822-831.
-
(2008)
Am J Respir Crit Care Med
, vol.178
, Issue.8
, pp. 822-831
-
-
Rowe, S.M.1
Jackson, P.L.2
Liu, G.3
-
55
-
-
0028914085
-
Early pulmonary inflammation in infants with cystic fibrosis
-
Khan T.Z., Wagener J.S., Bost T., et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995, 151(4):1075-1082.
-
(1995)
Am J Respir Crit Care Med
, vol.151
, Issue.4
, pp. 1075-1082
-
-
Khan, T.Z.1
Wagener, J.S.2
Bost, T.3
-
56
-
-
61949089083
-
Lipid metabolism in cystic fibrosis
-
Worgall T.S. Lipid metabolism in cystic fibrosis. Curr Opin Clin Nutr Metab Care 2009, 12(2):105-109.
-
(2009)
Curr Opin Clin Nutr Metab Care
, vol.12
, Issue.2
, pp. 105-109
-
-
Worgall, T.S.1
-
57
-
-
4644272735
-
Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway
-
Karp C.L., Flick L.M., Park K.W., et al. Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway. Nat Immunol 2004, 5(4):388-392.
-
(2004)
Nat Immunol
, vol.5
, Issue.4
, pp. 388-392
-
-
Karp, C.L.1
Flick, L.M.2
Park, K.W.3
-
58
-
-
84905657036
-
Reduced 15-lipoxygenase 2 and lipoxin A4/leukotriene B4 ratio in children with cystic fibrosis
-
Ringholz F.C., Buchanan P.J., Clarke D.T., et al. Reduced 15-lipoxygenase 2 and lipoxin A4/leukotriene B4 ratio in children with cystic fibrosis. Eur Respir J 2014, 44(2):394-404.
-
(2014)
Eur Respir J
, vol.44
, Issue.2
, pp. 394-404
-
-
Ringholz, F.C.1
Buchanan, P.J.2
Clarke, D.T.3
-
59
-
-
84884966507
-
Clinical effects of diet supplementation with DHA in pediatric patients suffering from cystic fibrosis
-
Leggieri E., De Biase R.V., Savi D., et al. Clinical effects of diet supplementation with DHA in pediatric patients suffering from cystic fibrosis. Minerva Pediatr 2013, 65(4):389-398.
-
(2013)
Minerva Pediatr
, vol.65
, Issue.4
, pp. 389-398
-
-
Leggieri, E.1
De Biase, R.V.2
Savi, D.3
-
60
-
-
47249102033
-
Tissue transglutaminase activation modulates inflammation in cystic fibrosis via PPARgamma down-regulation
-
Maiuri L., Luciani A., Giardino I., et al. Tissue transglutaminase activation modulates inflammation in cystic fibrosis via PPARgamma down-regulation. J Immunol 2008, 180(11):7697-7705.
-
(2008)
J Immunol
, vol.180
, Issue.11
, pp. 7697-7705
-
-
Maiuri, L.1
Luciani, A.2
Giardino, I.3
-
61
-
-
3042856185
-
Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice
-
Ollero M., Junaidi O., Zaman M.M., et al. Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice. J Cell Physiol 2004, 200(2):235-244.
-
(2004)
J Cell Physiol
, vol.200
, Issue.2
, pp. 235-244
-
-
Ollero, M.1
Junaidi, O.2
Zaman, M.M.3
-
62
-
-
54449093079
-
Dysfunction of Nrf-2 in CF epithelia leads to excess intracellular H2O2 and inflammatory cytokine production
-
Chen J., Kinter M., Shank S., et al. Dysfunction of Nrf-2 in CF epithelia leads to excess intracellular H2O2 and inflammatory cytokine production. PLoS One 2008, 3(10):e3367.
-
(2008)
PLoS One
, vol.3
, Issue.10
, pp. e3367
-
-
Chen, J.1
Kinter, M.2
Shank, S.3
-
63
-
-
79952668061
-
Role of endoplasmic reticulum stress in cystic fibrosis-related airway inflammatory responses
-
Ribeiro C.M., Boucher R.C. Role of endoplasmic reticulum stress in cystic fibrosis-related airway inflammatory responses. Proc Am Thorac Soc 2010, 7(6):387-394.
-
(2010)
Proc Am Thorac Soc
, vol.7
, Issue.6
, pp. 387-394
-
-
Ribeiro, C.M.1
Boucher, R.C.2
-
64
-
-
84855394399
-
Induction of type I interferon signaling by Pseudomonas aeruginosa is diminished in cystic fibrosis epithelial cells
-
Parker D., Cohen T.S., Alhede M., et al. Induction of type I interferon signaling by Pseudomonas aeruginosa is diminished in cystic fibrosis epithelial cells. Am J Respir Cell Mol Biol 2012, 46(1):6-13.
-
(2012)
Am J Respir Cell Mol Biol
, vol.46
, Issue.1
, pp. 6-13
-
-
Parker, D.1
Cohen, T.S.2
Alhede, M.3
-
65
-
-
0037728657
-
Impaired innate host defense causes susceptibility to respiratory virus infections in cystic fibrosis
-
Zheng S., De B.P., Choudhary S., et al. Impaired innate host defense causes susceptibility to respiratory virus infections in cystic fibrosis. Immunity 2003, 18(5):619-630.
-
(2003)
Immunity
, vol.18
, Issue.5
, pp. 619-630
-
-
Zheng, S.1
De, B.P.2
Choudhary, S.3
-
66
-
-
84876802220
-
Cystic fibrosis transmembrane conductance regulator recruitment to phagosomes in neutrophils
-
Zhou Y., Song K., Painter R.G., et al. Cystic fibrosis transmembrane conductance regulator recruitment to phagosomes in neutrophils. J Innate Immun 2013, 5(3):219-230.
-
(2013)
J Innate Immun
, vol.5
, Issue.3
, pp. 219-230
-
-
Zhou, Y.1
Song, K.2
Painter, R.G.3
-
67
-
-
80051663317
-
Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice
-
Su X., Looney M., Su H., et al. Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice. Inflamm Res 2011, 60(7):619-632.
-
(2011)
Inflamm Res
, vol.60
, Issue.7
, pp. 619-632
-
-
Su, X.1
Looney, M.2
Su, H.3
-
69
-
-
84929612761
-
Mature cystic fibrosis airway neutrophils suppress T cell function: evidence for a role of arginase 1 but not programmed death-ligand 1
-
Ingersoll S.A., Laval J., Forrest O.A., et al. Mature cystic fibrosis airway neutrophils suppress T cell function: evidence for a role of arginase 1 but not programmed death-ligand 1. J Immunol 2015, 194(11):5520-5528.
-
(2015)
J Immunol
, vol.194
, Issue.11
, pp. 5520-5528
-
-
Ingersoll, S.A.1
Laval, J.2
Forrest, O.A.3
-
70
-
-
80355131976
-
Protective and pathogenic functions of macrophage subsets
-
Murray P.J., Wynn T.A. Protective and pathogenic functions of macrophage subsets. Nat Rev Immunol 2011, 11(11):723-737.
-
(2011)
Nat Rev Immunol
, vol.11
, Issue.11
, pp. 723-737
-
-
Murray, P.J.1
Wynn, T.A.2
-
71
-
-
70349095833
-
Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis
-
Brennan S., Sly P.D., Gangell C.L., et al. Alveolar macrophages and CC chemokines are increased in children with cystic fibrosis. Eur Respir J 2009, 34(3):655-661.
-
(2009)
Eur Respir J
, vol.34
, Issue.3
, pp. 655-661
-
-
Brennan, S.1
Sly, P.D.2
Gangell, C.L.3
-
72
-
-
84879103109
-
Macrophage activation and polarization as an adaptive component of innate immunity
-
Locati M., Mantovani A., Sica A. Macrophage activation and polarization as an adaptive component of innate immunity. Adv Immunol 2013, 120:163-184.
-
(2013)
Adv Immunol
, vol.120
, pp. 163-184
-
-
Locati, M.1
Mantovani, A.2
Sica, A.3
-
73
-
-
33846907545
-
Alpha1-Antitrypsin inhalation reduces airway inflammation in cystic fibrosis patients
-
Griese M., Latzin P., Kappler M., et al. alpha1-Antitrypsin inhalation reduces airway inflammation in cystic fibrosis patients. Eur Respir J 2007, 29(2):240-250.
-
(2007)
Eur Respir J
, vol.29
, Issue.2
, pp. 240-250
-
-
Griese, M.1
Latzin, P.2
Kappler, M.3
-
74
-
-
70149123741
-
Azithromycin reduces exaggerated cytokine production by M1 alveolar macrophages in cystic fibrosis
-
Meyer M., Huaux F., Gavilanes X., et al. Azithromycin reduces exaggerated cytokine production by M1 alveolar macrophages in cystic fibrosis. Am J Respir Cell Mol Biol 2009, 41(5):590-602.
-
(2009)
Am J Respir Cell Mol Biol
, vol.41
, Issue.5
, pp. 590-602
-
-
Meyer, M.1
Huaux, F.2
Gavilanes, X.3
-
75
-
-
84913555523
-
Decreased expression of HLA-DQ and HLA-DR on cells of the monocytic lineage in cystic fibrosis
-
Hofer T.P., Frankenberger M., Heimbeck I., et al. Decreased expression of HLA-DQ and HLA-DR on cells of the monocytic lineage in cystic fibrosis. J Mol Med (Berl) 2014, 92(12):1293-1304.
-
(2014)
J Mol Med (Berl)
, vol.92
, Issue.12
, pp. 1293-1304
-
-
Hofer, T.P.1
Frankenberger, M.2
Heimbeck, I.3
-
76
-
-
84952039026
-
X-Box binding protein 1 modulates innate immune responses of cystic fibrosis alveolar macrophages
-
[Epub ahead of print]
-
Lubamba B.A., Jones L.C., O'Neal W.K., et al. X-Box binding protein 1 modulates innate immune responses of cystic fibrosis alveolar macrophages. Am J Respir Crit Care Med 2015, [Epub ahead of print].
-
(2015)
Am J Respir Crit Care Med
-
-
Lubamba, B.A.1
Jones, L.C.2
O'Neal, W.K.3
-
77
-
-
4644313798
-
Interleukin 8 secretion from monocytes of subjects heterozygous for the deltaF508 cystic fibrosis transmembrane conductance regulator gene mutation is altered
-
Zaman M.M., Gelrud A., Junaidi O., et al. Interleukin 8 secretion from monocytes of subjects heterozygous for the deltaF508 cystic fibrosis transmembrane conductance regulator gene mutation is altered. Clin Diagn Lab Immunol 2004, 11(5):819-824.
-
(2004)
Clin Diagn Lab Immunol
, vol.11
, Issue.5
, pp. 819-824
-
-
Zaman, M.M.1
Gelrud, A.2
Junaidi, O.3
-
78
-
-
38049091190
-
Alterations in immune response and PPAR/LXR regulation in cystic fibrosis macrophages
-
Andersson C., Zaman M.M., Jones A.B., et al. Alterations in immune response and PPAR/LXR regulation in cystic fibrosis macrophages. J Cyst Fibros 2008, 7(1):68-78.
-
(2008)
J Cyst Fibros
, vol.7
, Issue.1
, pp. 68-78
-
-
Andersson, C.1
Zaman, M.M.2
Jones, A.B.3
-
79
-
-
84868322168
-
Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection
-
Bonfield T.L., Hodges C.A., Cotton C.U., et al. Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection. J Leukoc Biol 2012, 92(5):1111-1122.
-
(2012)
J Leukoc Biol
, vol.92
, Issue.5
, pp. 1111-1122
-
-
Bonfield, T.L.1
Hodges, C.A.2
Cotton, C.U.3
-
80
-
-
84866182329
-
Innate immunity in cystic fibrosis lung disease
-
Hartl D., Gaggar A., Bruscia E., et al. Innate immunity in cystic fibrosis lung disease. J Cyst Fibros 2012, 11(5):363-382.
-
(2012)
J Cyst Fibros
, vol.11
, Issue.5
, pp. 363-382
-
-
Hartl, D.1
Gaggar, A.2
Bruscia, E.3
-
81
-
-
79957588506
-
Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2
-
Wright F.A., Strug L.J., Doshi V.K., et al. Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2. Nat Genet 2011, 43(6):539-546.
-
(2011)
Nat Genet
, vol.43
, Issue.6
, pp. 539-546
-
-
Wright, F.A.1
Strug, L.J.2
Doshi, V.K.3
-
82
-
-
0032935226
-
HLA class II polymorphism in cystic fibrosis. A possible modifier of pulmonary phenotype
-
Aron Y., Polla B.S., Bienvenu T., et al. HLA class II polymorphism in cystic fibrosis. A possible modifier of pulmonary phenotype. Am J Respir Crit Care Med 1999, 159(5 Pt 1):1464-1468.
-
(1999)
Am J Respir Crit Care Med
, vol.159
, Issue.5
, pp. 1464-1468
-
-
Aron, Y.1
Polla, B.S.2
Bienvenu, T.3
-
84
-
-
84861877852
-
Immunopathology and immunogenetics of allergic bronchopulmonary aspergillosis
-
Knutsen A.P. Immunopathology and immunogenetics of allergic bronchopulmonary aspergillosis. J Allergy (Cairo) 2011, 2011:785983.
-
(2011)
J Allergy (Cairo)
, vol.2011
, pp. 785983
-
-
Knutsen, A.P.1
-
85
-
-
34548261376
-
HLA-DRB1 alleles control allergic bronchopulmonary aspergillosis-like pulmonary responses in humanized transgenic mice
-
Koehm S., Slavin R.G., Hutcheson P.S., et al. HLA-DRB1 alleles control allergic bronchopulmonary aspergillosis-like pulmonary responses in humanized transgenic mice. J Allergy Clin Immunol 2007, 120(3):570-577.
-
(2007)
J Allergy Clin Immunol
, vol.120
, Issue.3
, pp. 570-577
-
-
Koehm, S.1
Slavin, R.G.2
Hutcheson, P.S.3
-
86
-
-
80655134731
-
Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis
-
Abdulrahman B.A., Khweek A.A., Akhter A., et al. Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis. Autophagy 2011, 7(11):1359-1370.
-
(2011)
Autophagy
, vol.7
, Issue.11
, pp. 1359-1370
-
-
Abdulrahman, B.A.1
Khweek, A.A.2
Akhter, A.3
-
87
-
-
84880897081
-
Interleukin-33 drives activation of alveolar macrophages and airway inflammation in a mouse model of acute exacerbation of chronic asthma
-
Bunting M.M., Shadie A.M., Flesher R.P., et al. Interleukin-33 drives activation of alveolar macrophages and airway inflammation in a mouse model of acute exacerbation of chronic asthma. Biomed Res Int 2013, 2013:250938.
-
(2013)
Biomed Res Int
, vol.2013
, pp. 250938
-
-
Bunting, M.M.1
Shadie, A.M.2
Flesher, R.P.3
-
88
-
-
0031041037
-
Defective antigen presentation by lavage cells from terminal patients with cystic fibrosis
-
Knight R.A., Kollnberger S., Madden B., et al. Defective antigen presentation by lavage cells from terminal patients with cystic fibrosis. Clin Exp Immunol 1997, 107(3):542-547.
-
(1997)
Clin Exp Immunol
, vol.107
, Issue.3
, pp. 542-547
-
-
Knight, R.A.1
Kollnberger, S.2
Madden, B.3
-
89
-
-
77950333055
-
A novel approach to analyze gene expression data demonstrates that the DeltaF508 mutation in CFTR downregulates the antigen presentation pathway
-
Hampton T.H., Stanton B.A. A novel approach to analyze gene expression data demonstrates that the DeltaF508 mutation in CFTR downregulates the antigen presentation pathway. Am J Physiol Lung Cell Mol Physiol 2010, 298(4):L473-L482.
-
(2010)
Am J Physiol Lung Cell Mol Physiol
, vol.298
, Issue.4
, pp. L473-L482
-
-
Hampton, T.H.1
Stanton, B.A.2
-
90
-
-
84882911145
-
Toll like receptor (TLR)-induced differential expression of microRNAs (MiRs) promotes proper immune response against infections: a systematic review
-
Aalaei-Andabili S.H., Rezaei N. Toll like receptor (TLR)-induced differential expression of microRNAs (MiRs) promotes proper immune response against infections: a systematic review. J Infect 2013, 67(4):251-264.
-
(2013)
J Infect
, vol.67
, Issue.4
, pp. 251-264
-
-
Aalaei-Andabili, S.H.1
Rezaei, N.2
-
91
-
-
84873386239
-
Low sphingosine-1-phosphate impairs lung dendritic cells in cystic fibrosis
-
Xu Y., Krause A., Limberis M., et al. Low sphingosine-1-phosphate impairs lung dendritic cells in cystic fibrosis. Am J Respir Cell Mol Biol 2013, 48(2):250-257.
-
(2013)
Am J Respir Cell Mol Biol
, vol.48
, Issue.2
, pp. 250-257
-
-
Xu, Y.1
Krause, A.2
Limberis, M.3
-
92
-
-
23944497169
-
Decreased apoptosis in polyamine depleted IEC-6 cells depends on Akt-mediated NF-kappaB activation but not GSK3beta activity
-
Bhattacharya S., Ray R.M., Johnson L.R. Decreased apoptosis in polyamine depleted IEC-6 cells depends on Akt-mediated NF-kappaB activation but not GSK3beta activity. Apoptosis 2005, 10(4):759-776.
-
(2005)
Apoptosis
, vol.10
, Issue.4
, pp. 759-776
-
-
Bhattacharya, S.1
Ray, R.M.2
Johnson, L.R.3
-
93
-
-
84873721377
-
Edible blue-green algae reduce the production of pro-inflammatory cytokines by inhibiting NF-kappaB pathway in macrophages and splenocytes
-
Ku C.S., Pham T.X., Park Y., et al. Edible blue-green algae reduce the production of pro-inflammatory cytokines by inhibiting NF-kappaB pathway in macrophages and splenocytes. Biochim Biophys Acta 2013, 1830(4):2981-2988.
-
(2013)
Biochim Biophys Acta
, vol.1830
, Issue.4
, pp. 2981-2988
-
-
Ku, C.S.1
Pham, T.X.2
Park, Y.3
-
94
-
-
84875225940
-
Corticosteroid resistance in patients with asthma and chronic obstructive pulmonary disease
-
Barnes P.J. Corticosteroid resistance in patients with asthma and chronic obstructive pulmonary disease. J Allergy Clin Immunol 2013, 131(3):636-645.
-
(2013)
J Allergy Clin Immunol
, vol.131
, Issue.3
, pp. 636-645
-
-
Barnes, P.J.1
-
95
-
-
84864018748
-
The HDAC inhibitor Givinostat modulates the hematopoietic transcription factors NFE2 and C-MYB in JAK2(V617F) myeloproliferative neoplasm cells
-
Amaru Calzada A., Todoerti K., Donadoni L., et al. The HDAC inhibitor Givinostat modulates the hematopoietic transcription factors NFE2 and C-MYB in JAK2(V617F) myeloproliferative neoplasm cells. Exp Hematol 2012, 40(8):634-645.e10.
-
(2012)
Exp Hematol
, vol.40
, Issue.8
, pp. 634-645.e10
-
-
Amaru Calzada, A.1
Todoerti, K.2
Donadoni, L.3
-
97
-
-
77954013043
-
Differential effects of selective HDAC inhibitors on macrophage inflammatory responses to the Toll-like receptor 4 agonist LPS
-
Halili M.A., Andrews M.R., Labzin L.I., et al. Differential effects of selective HDAC inhibitors on macrophage inflammatory responses to the Toll-like receptor 4 agonist LPS. J Leukoc Biol 2010, 87(6):1103-1114.
-
(2010)
J Leukoc Biol
, vol.87
, Issue.6
, pp. 1103-1114
-
-
Halili, M.A.1
Andrews, M.R.2
Labzin, L.I.3
-
98
-
-
1842684989
-
HDAC6 deacetylase activity links the tubulin cytoskeleton with immune synapse organization
-
Serrador J.M., Cabrero J.R., Sancho D., et al. HDAC6 deacetylase activity links the tubulin cytoskeleton with immune synapse organization. Immunity 2004, 20(4):417-428.
-
(2004)
Immunity
, vol.20
, Issue.4
, pp. 417-428
-
-
Serrador, J.M.1
Cabrero, J.R.2
Sancho, D.3
-
99
-
-
58149359833
-
Oxidative stress causes IL8 promoter hyperacetylation in cystic fibrosis airway cell models
-
Bartling T.R., Drumm M.L. Oxidative stress causes IL8 promoter hyperacetylation in cystic fibrosis airway cell models. Am J Respir Cell Mol Biol 2009, 40(1):58-65.
-
(2009)
Am J Respir Cell Mol Biol
, vol.40
, Issue.1
, pp. 58-65
-
-
Bartling, T.R.1
Drumm, M.L.2
-
100
-
-
67650073195
-
Loss of CFTR results in reduction of histone deacetylase 2 in airway epithelial cells
-
Bartling T.R., Drumm M.L. Loss of CFTR results in reduction of histone deacetylase 2 in airway epithelial cells. Am J Physiol Lung Cell Mol Physiol 2009, 297(1):L35-L43.
-
(2009)
Am J Physiol Lung Cell Mol Physiol
, vol.297
, Issue.1
, pp. L35-L43
-
-
Bartling, T.R.1
Drumm, M.L.2
-
101
-
-
13444312010
-
Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice
-
Saadane A., Soltys J., Berger M. Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice. J Allergy Clin Immunol 2005, 115(2):405-411.
-
(2005)
J Allergy Clin Immunol
, vol.115
, Issue.2
, pp. 405-411
-
-
Saadane, A.1
Soltys, J.2
Berger, M.3
-
102
-
-
33847025695
-
CFTR inhibition mimics the cystic fibrosis inflammatory profile
-
Perez A., Issler A.C., Cotton C.U., et al. CFTR inhibition mimics the cystic fibrosis inflammatory profile. Am J Physiol Lung Cell Mol Physiol 2007, 292(2):L383-L395.
-
(2007)
Am J Physiol Lung Cell Mol Physiol
, vol.292
, Issue.2
, pp. L383-L395
-
-
Perez, A.1
Issler, A.C.2
Cotton, C.U.3
-
103
-
-
84863359416
-
Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis
-
Regamey N., Tsartsali L., Hilliard T.N., et al. Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis. Thorax 2012, 67(2):164-170.
-
(2012)
Thorax
, vol.67
, Issue.2
, pp. 164-170
-
-
Regamey, N.1
Tsartsali, L.2
Hilliard, T.N.3
-
104
-
-
80051553123
-
The Th17 pathway in cystic fibrosis lung disease
-
Tan H.L., Regamey N., Brown S., et al. The Th17 pathway in cystic fibrosis lung disease. Am J Respir Crit Care Med 2011, 184(2):252-258.
-
(2011)
Am J Respir Crit Care Med
, vol.184
, Issue.2
, pp. 252-258
-
-
Tan, H.L.1
Regamey, N.2
Brown, S.3
-
105
-
-
29544436136
-
Pulmonary T(H)2 response in Pseudomonas aeruginosa-infected patients with cystic fibrosis
-
Hartl D., Griese M., Kappler M., et al. Pulmonary T(H)2 response in Pseudomonas aeruginosa-infected patients with cystic fibrosis. J Allergy Clin Immunol 2006, 117(1):204-211.
-
(2006)
J Allergy Clin Immunol
, vol.117
, Issue.1
, pp. 204-211
-
-
Hartl, D.1
Griese, M.2
Kappler, M.3
-
106
-
-
84927794362
-
Regulatory T cell impairment in cystic fibrosis patients with chronic Pseudomonas infection
-
Hector A., Schafer H., Poschel S., et al. Regulatory T cell impairment in cystic fibrosis patients with chronic Pseudomonas infection. Am J Respir Crit Care Med 2015, 191(8):914-923.
-
(2015)
Am J Respir Crit Care Med
, vol.191
, Issue.8
, pp. 914-923
-
-
Hector, A.1
Schafer, H.2
Poschel, S.3
-
107
-
-
77956407606
-
Vitamin D3 attenuates Th2 responses to Aspergillus fumigatus mounted by CD4+ T cells from cystic fibrosis patients with allergic bronchopulmonary aspergillosis
-
Kreindler J.L., Steele C., Nguyen N., et al. Vitamin D3 attenuates Th2 responses to Aspergillus fumigatus mounted by CD4+ T cells from cystic fibrosis patients with allergic bronchopulmonary aspergillosis. J Clin Invest 2010, 120(9):3242-3254.
-
(2010)
J Clin Invest
, vol.120
, Issue.9
, pp. 3242-3254
-
-
Kreindler, J.L.1
Steele, C.2
Nguyen, N.3
-
108
-
-
84927794365
-
Regulatory T cells in cystic fibrosis lung disease. More answers, more questions
-
McGuire J.K. Regulatory T cells in cystic fibrosis lung disease. More answers, more questions. Am J Respir Crit Care Med 2015, 191(8):866-868.
-
(2015)
Am J Respir Crit Care Med
, vol.191
, Issue.8
, pp. 866-868
-
-
McGuire, J.K.1
-
109
-
-
84875158201
-
Th17/Treg imbalance in murine cystic fibrosis is linked to indoleamine 2,3-dioxygenase deficiency but corrected by kynurenines
-
Iannitti R.G., Carvalho A., Cunha C., et al. Th17/Treg imbalance in murine cystic fibrosis is linked to indoleamine 2,3-dioxygenase deficiency but corrected by kynurenines. Am J Respir Crit Care Med 2013, 187(6):609-620.
-
(2013)
Am J Respir Crit Care Med
, vol.187
, Issue.6
, pp. 609-620
-
-
Iannitti, R.G.1
Carvalho, A.2
Cunha, C.3
-
110
-
-
80051554943
-
Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses
-
Mueller C., Braag S.A., Keeler A., et al. Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses. Am J Respir Cell Mol Biol 2011, 44(6):922-929.
-
(2011)
Am J Respir Cell Mol Biol
, vol.44
, Issue.6
, pp. 922-929
-
-
Mueller, C.1
Braag, S.A.2
Keeler, A.3
-
111
-
-
0029849044
-
Reduced IL-10 secretion by CD4+ T lymphocytes expressing mutant cystic fibrosis transmembrane conductance regulator (CFTR)
-
Moss R.B., Bocian R.C., Hsu Y.P., et al. Reduced IL-10 secretion by CD4+ T lymphocytes expressing mutant cystic fibrosis transmembrane conductance regulator (CFTR). Clin Exp Immunol 1996, 106(2):374-388.
-
(1996)
Clin Exp Immunol
, vol.106
, Issue.2
, pp. 374-388
-
-
Moss, R.B.1
Bocian, R.C.2
Hsu, Y.P.3
-
112
-
-
0037083448
-
Functional IL-10 deficiency in the lung of cystic fibrosis (cftr(-/-)) and IL-10 knockout mice causes increased expression and function of B7 costimulatory molecules on alveolar macrophages
-
Soltys J., Bonfield T., Chmiel J., et al. Functional IL-10 deficiency in the lung of cystic fibrosis (cftr(-/-)) and IL-10 knockout mice causes increased expression and function of B7 costimulatory molecules on alveolar macrophages. J Immunol 2002, 168(4):1903-1910.
-
(2002)
J Immunol
, vol.168
, Issue.4
, pp. 1903-1910
-
-
Soltys, J.1
Bonfield, T.2
Chmiel, J.3
-
113
-
-
34247390062
-
Association between pulmonary function and sputum biomarkers in cystic fibrosis
-
Mayer-Hamblett N., Aitken M.L., Accurso F.J., et al. Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med 2007, 175(8):822-828.
-
(2007)
Am J Respir Crit Care Med
, vol.175
, Issue.8
, pp. 822-828
-
-
Mayer-Hamblett, N.1
Aitken, M.L.2
Accurso, F.J.3
-
114
-
-
84895069337
-
A randomized double blind, placebo controlled phase 2 trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis
-
Konstan M.W., Doring G., Heltshe S.L., Investigators and Coordinators of BI Trial 543.45, et al. A randomized double blind, placebo controlled phase 2 trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis. J Cyst Fibros 2014, 13(2):148-155.
-
(2014)
J Cyst Fibros
, vol.13
, Issue.2
, pp. 148-155
-
-
Konstan, M.W.1
Doring, G.2
Heltshe, S.L.3
-
116
-
-
65349121194
-
IL-17-producing T cells in lung immunity and inflammation
-
[quiz: 995-6]
-
Nembrini C., Marsland B.J., Kopf M. IL-17-producing T cells in lung immunity and inflammation. J Allergy Clin Immunol 2009, 123(5):986-994. [quiz: 995-6].
-
(2009)
J Allergy Clin Immunol
, vol.123
, Issue.5
, pp. 986-994
-
-
Nembrini, C.1
Marsland, B.J.2
Kopf, M.3
-
117
-
-
0034807936
-
Interleukin-17 and lung host defense against Klebsiella pneumoniae infection
-
Ye P., Garvey P.B., Zhang P., et al. Interleukin-17 and lung host defense against Klebsiella pneumoniae infection. Am J Respir Cell Mol Biol 2001, 25(3):335-340.
-
(2001)
Am J Respir Cell Mol Biol
, vol.25
, Issue.3
, pp. 335-340
-
-
Ye, P.1
Garvey, P.B.2
Zhang, P.3
-
118
-
-
0037371262
-
IL-17 promotes bone erosion in murine collagen-induced arthritis through loss of the receptor activator of NF-kappa B ligand/osteoprotegerin balance
-
Lubberts E., van den Bersselaar L., Oppers-Walgreen B., et al. IL-17 promotes bone erosion in murine collagen-induced arthritis through loss of the receptor activator of NF-kappa B ligand/osteoprotegerin balance. J Immunol 2003, 170(5):2655-2662.
-
(2003)
J Immunol
, vol.170
, Issue.5
, pp. 2655-2662
-
-
Lubberts, E.1
van den Bersselaar, L.2
Oppers-Walgreen, B.3
-
119
-
-
79551592779
-
Elevated expression of both mRNA and protein levels of IL-17A in sputum of stable cystic fibrosis patients
-
Decraene A., Willems-Widyastuti A., Kasran A., et al. Elevated expression of both mRNA and protein levels of IL-17A in sputum of stable cystic fibrosis patients. Respir Res 2010, 11:177.
-
(2010)
Respir Res
, vol.11
, pp. 177
-
-
Decraene, A.1
Willems-Widyastuti, A.2
Kasran, A.3
-
120
-
-
84879939404
-
IL-17 in lung disease: friend or foe?
-
Tan H.L., Rosenthal M. IL-17 in lung disease: friend or foe?. Thorax 2013, 68(8):788-790.
-
(2013)
Thorax
, vol.68
, Issue.8
, pp. 788-790
-
-
Tan, H.L.1
Rosenthal, M.2
-
121
-
-
84875712531
-
Patients with cystic fibrosis have inducible IL-17+IL-22+ memory cells in lung draining lymph nodes
-
1129.e1-5
-
Chan Y.R., Chen K., Duncan S.R., et al. Patients with cystic fibrosis have inducible IL-17+IL-22+ memory cells in lung draining lymph nodes. J Allergy Clin Immunol 2013, 131(4):1117-1129. 1129.e1-5.
-
(2013)
J Allergy Clin Immunol
, vol.131
, Issue.4
, pp. 1117-1129
-
-
Chan, Y.R.1
Chen, K.2
Duncan, S.R.3
-
122
-
-
84856560323
-
IL-13 regulates Th17 secretion of IL-17A in an IL-10-dependent manner
-
Newcomb D.C., Boswell M.G., Huckabee M.M., et al. IL-13 regulates Th17 secretion of IL-17A in an IL-10-dependent manner. J Immunol 2012, 188(3):1027-1035.
-
(2012)
J Immunol
, vol.188
, Issue.3
, pp. 1027-1035
-
-
Newcomb, D.C.1
Boswell, M.G.2
Huckabee, M.M.3
-
123
-
-
77951861324
-
IL-17 is essential for host defense against cutaneous Staphylococcus aureus infection in mice
-
Cho J.S., Pietras E.M., Garcia N.C., et al. IL-17 is essential for host defense against cutaneous Staphylococcus aureus infection in mice. J Clin Invest 2010, 120(5):1762-1773.
-
(2010)
J Clin Invest
, vol.120
, Issue.5
, pp. 1762-1773
-
-
Cho, J.S.1
Pietras, E.M.2
Garcia, N.C.3
-
124
-
-
84903989532
-
Invariant natural killer T (iNKT) cells prevent autoimmunity, but induce pulmonary inflammation in cystic fibrosis
-
Siegmann N., Worbs D., Effinger F., et al. Invariant natural killer T (iNKT) cells prevent autoimmunity, but induce pulmonary inflammation in cystic fibrosis. Cell Physiol Biochem 2014, 34(1):56-70.
-
(2014)
Cell Physiol Biochem
, vol.34
, Issue.1
, pp. 56-70
-
-
Siegmann, N.1
Worbs, D.2
Effinger, F.3
-
125
-
-
36848999782
-
Clinical use of ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis
-
Konstan M.W., Schluchter M.D., Xue W., et al. Clinical use of ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis. Am J Respir Crit Care Med 2007, 176(11):1084-1089.
-
(2007)
Am J Respir Crit Care Med
, vol.176
, Issue.11
, pp. 1084-1089
-
-
Konstan, M.W.1
Schluchter, M.D.2
Xue, W.3
-
126
-
-
84875829440
-
Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health
-
Mogayzel P.J., Naureckas E.T., Robinson K.A., Pulmonary Clinical Practice Guidelines Committee, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2013, 187(7):680-689.
-
(2013)
Am J Respir Crit Care Med
, vol.187
, Issue.7
, pp. 680-689
-
-
Mogayzel, P.J.1
Naureckas, E.T.2
Robinson, K.A.3
-
127
-
-
84947038442
-
Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation
-
Zhang P.X., Cheng J., Zou S., et al. Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation. Nat Commun 2015, 6:6221.
-
(2015)
Nat Commun
, vol.6
, pp. 6221
-
-
Zhang, P.X.1
Cheng, J.2
Zou, S.3
-
128
-
-
0028969594
-
A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group
-
Eigen H., Rosenstein B.J., FitzSimmons S., et al. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr 1995, 126(4):515-523.
-
(1995)
J Pediatr
, vol.126
, Issue.4
, pp. 515-523
-
-
Eigen, H.1
Rosenstein, B.J.2
FitzSimmons, S.3
-
129
-
-
62949240359
-
The role of inhaled corticosteroids in the management of cystic fibrosis
-
Ross K.R., Chmiel J.F., Konstan M.W. The role of inhaled corticosteroids in the management of cystic fibrosis. Paediatr Drugs 2009, 11(2):101-113.
-
(2009)
Paediatr Drugs
, vol.11
, Issue.2
, pp. 101-113
-
-
Ross, K.R.1
Chmiel, J.F.2
Konstan, M.W.3
-
130
-
-
84886091119
-
A pilot study evaluating the effect of pioglitazone, simvastatin and ibuprofen on neutrophil migration in vivo in healthy subjects
-
Konstan M., Krenicky J., Hilliard K., et al. A pilot study evaluating the effect of pioglitazone, simvastatin and ibuprofen on neutrophil migration in vivo in healthy subjects. Pediatr Pulmonol 2009, 289-290.
-
(2009)
Pediatr Pulmonol
, pp. 289-290
-
-
Konstan, M.1
Krenicky, J.2
Hilliard, K.3
-
131
-
-
20144387174
-
Randomized, double-blind, placebo-controlled, dose-escalating study of aerosolized interferon gamma-1b in patients with mild to moderate cystic fibrosis lung disease
-
Moss R.B., Mayer-Hamblett N., Wagener J., et al. Randomized, double-blind, placebo-controlled, dose-escalating study of aerosolized interferon gamma-1b in patients with mild to moderate cystic fibrosis lung disease. Pediatr Pulmonol 2005, 39(3):209-218.
-
(2005)
Pediatr Pulmonol
, vol.39
, Issue.3
, pp. 209-218
-
-
Moss, R.B.1
Mayer-Hamblett, N.2
Wagener, J.3
-
132
-
-
13844322148
-
A pilot study of the effect of inhaled buffered reduced glutathione on the clinical status of patients with cystic fibrosis
-
Bishop C., Hudson V.M., Hilton S.C., et al. A pilot study of the effect of inhaled buffered reduced glutathione on the clinical status of patients with cystic fibrosis. Chest 2005, 127(1):308-317.
-
(2005)
Chest
, vol.127
, Issue.1
, pp. 308-317
-
-
Bishop, C.1
Hudson, V.M.2
Hilton, S.C.3
-
133
-
-
22544453909
-
Inhaled glutathione decreases PGE2 and increases lymphocytes in cystic fibrosis lungs
-
Hartl D., Starosta V., Maier K., et al. Inhaled glutathione decreases PGE2 and increases lymphocytes in cystic fibrosis lungs. Free Radic Biol Med 2005, 39(4):463-472.
-
(2005)
Free Radic Biol Med
, vol.39
, Issue.4
, pp. 463-472
-
-
Hartl, D.1
Starosta, V.2
Maier, K.3
-
134
-
-
84924340525
-
Long-term treatment with oral N-acetylcysteine: affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial
-
Conrad C., Lymp J., Thompson V., et al. Long-term treatment with oral N-acetylcysteine: affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial. J Cyst Fibros 2015, 14(2):219-227.
-
(2015)
J Cyst Fibros
, vol.14
, Issue.2
, pp. 219-227
-
-
Conrad, C.1
Lymp, J.2
Thompson, V.3
-
135
-
-
84876987955
-
Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis
-
Moss R.B., Mistry S.J., Konstan M.W., et al. Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis. J Cyst Fibros 2013, 12(3):241-248.
-
(2013)
J Cyst Fibros
, vol.12
, Issue.3
, pp. 241-248
-
-
Moss, R.B.1
Mistry, S.J.2
Konstan, M.W.3
-
136
-
-
84977457103
-
Inhaled alpha-proteinase inhibitor therapy in patients with cystic fibrosis
-
[Epub ahead of print]
-
Gaggar A., Chen J., Chmiel J.F., et al. Inhaled alpha-proteinase inhibitor therapy in patients with cystic fibrosis. J Cyst Fibros 2015, [Epub ahead of print].
-
(2015)
J Cyst Fibros
-
-
Gaggar, A.1
Chen, J.2
Chmiel, J.F.3
-
137
-
-
79551607386
-
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury
-
Gould N.S., Gauthier S., Kariya C.T., et al. Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury. Respir Res 2010, 11:119.
-
(2010)
Respir Res
, vol.11
, pp. 119
-
-
Gould, N.S.1
Gauthier, S.2
Kariya, C.T.3
-
138
-
-
84938928408
-
Antiinflammatory and antimicrobial effects of thiocyanate in a cystic fibrosis mouse model
-
Chandler J.D., Min E., Huang J., et al. Antiinflammatory and antimicrobial effects of thiocyanate in a cystic fibrosis mouse model. Am J Respir Cell Mol Biol 2015, 53(2):193-205.
-
(2015)
Am J Respir Cell Mol Biol
, vol.53
, Issue.2
, pp. 193-205
-
-
Chandler, J.D.1
Min, E.2
Huang, J.3
-
139
-
-
13444292529
-
Oral L-arginine supplementation in cystic fibrosis patients: a placebo-controlled study
-
Grasemann H., Grasemann C., Kurtz F., et al. Oral L-arginine supplementation in cystic fibrosis patients: a placebo-controlled study. Eur Respir J 2005, 25(1):62-68.
-
(2005)
Eur Respir J
, vol.25
, Issue.1
, pp. 62-68
-
-
Grasemann, H.1
Grasemann, C.2
Kurtz, F.3
-
140
-
-
72449174784
-
The triterpenoid CDDO limits inflammation in preclinical models of cystic fibrosis lung disease
-
Nichols D.P., Ziady A.G., Shank S.L., et al. The triterpenoid CDDO limits inflammation in preclinical models of cystic fibrosis lung disease. Am J Physiol Lung Cell Mol Physiol 2009, 297(5):L828-L836.
-
(2009)
Am J Physiol Lung Cell Mol Physiol
, vol.297
, Issue.5
, pp. L828-L836
-
-
Nichols, D.P.1
Ziady, A.G.2
Shank, S.L.3
-
141
-
-
84922575896
-
Lipid mediators in the resolution of inflammation
-
Serhan C.N., Chiang N., Dalli J., et al. Lipid mediators in the resolution of inflammation. Cold Spring Harb Perspect Biol 2015, 7(2):a016311.
-
(2015)
Cold Spring Harb Perspect Biol
, vol.7
, Issue.2
, pp. a016311
-
-
Serhan, C.N.1
Chiang, N.2
Dalli, J.3
-
142
-
-
0027287651
-
Efficacy of aerosolized tobramycin in patients with cystic fibrosis
-
Ramsey B.W., Dorkin H.L., Eisenberg J.D., et al. Efficacy of aerosolized tobramycin in patients with cystic fibrosis. N Engl J Med 1993, 328(24):1740-1746.
-
(1993)
N Engl J Med
, vol.328
, Issue.24
, pp. 1740-1746
-
-
Ramsey, B.W.1
Dorkin, H.L.2
Eisenberg, J.D.3
-
143
-
-
84866076450
-
Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa
-
Ratjen F., Saiman L., Mayer-Hamblett N., et al. Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa. Chest 2012, 142(5):1259-1266.
-
(2012)
Chest
, vol.142
, Issue.5
, pp. 1259-1266
-
-
Ratjen, F.1
Saiman, L.2
Mayer-Hamblett, N.3
-
144
-
-
84862179762
-
Open-label, follow-on study of azithromycin in pediatric patients with CF uninfected with Pseudomonas aeruginosa
-
Saiman L., Mayer-Hamblett N., Anstead M., AZ0004 Macrolide Study Team, et al. Open-label, follow-on study of azithromycin in pediatric patients with CF uninfected with Pseudomonas aeruginosa. Pediatr Pulmonol 2012, 47(7):641-648.
-
(2012)
Pediatr Pulmonol
, vol.47
, Issue.7
, pp. 641-648
-
-
Saiman, L.1
Mayer-Hamblett, N.2
Anstead, M.3
-
145
-
-
77951887288
-
Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial
-
Saiman L., Anstead M., Mayer-Hamblett N., AZ0004 Azithromycin Study Group, et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 2010, 303(17):1707-1715.
-
(2010)
JAMA
, vol.303
, Issue.17
, pp. 1707-1715
-
-
Saiman, L.1
Anstead, M.2
Mayer-Hamblett, N.3
-
146
-
-
84937631483
-
Inflammation in cystic fibrosis lung disease: pathogenesis and therapy
-
Cantin A.M., Hartl D., Konstan M.W., et al. Inflammation in cystic fibrosis lung disease: pathogenesis and therapy. J Cyst Fibros 2015, 14(4):419-430.
-
(2015)
J Cyst Fibros
, vol.14
, Issue.4
, pp. 419-430
-
-
Cantin, A.M.1
Hartl, D.2
Konstan, M.W.3
-
147
-
-
78549279173
-
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation
-
Accurso F.J., Rowe S.M., Clancy J.P., et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 2010, 363(21):1991-2003.
-
(2010)
N Engl J Med
, vol.363
, Issue.21
, pp. 1991-2003
-
-
Accurso, F.J.1
Rowe, S.M.2
Clancy, J.P.3
-
148
-
-
84937035647
-
Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR
-
Wainwright C.E., Elborn J.S., Ramsey B.W., TRAFFIC Study Group, TRANSPORT Study Group, et al. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med 2015, 373(3):220-231.
-
(2015)
N Engl J Med
, vol.373
, Issue.3
, pp. 220-231
-
-
Wainwright, C.E.1
Elborn, J.S.2
Ramsey, B.W.3
|