-
1
-
-
0021957142
-
Prenatal diagnosis of cystic fibrosis. II. Meconium ileus in affected fetuses
-
Muller F, Aubry MC, Gasser B, Duchatel F, Boué J, Boué A. Prenatal diagnosis of cystic fibrosis. II. Meconium ileus in affected fetuses. Prenatal Diagn. 5(2), 109-117 (1985).
-
(1985)
Prenatal Diagn.
, vol.5
, Issue.2
, pp. 109-117
-
-
Muller, F.1
Aubry, M.C.2
Gasser, B.3
Duchatel, F.4
Boué, J.5
Boué, A.6
-
2
-
-
84894056957
-
Gastrointestinal surgery in cystic fibrosis: A 20-year review
-
Farrelly PJ, Charlesworth C, Lee S, Southern KW, Baillie CT. Gastrointestinal surgery in cystic fibrosis: a 20-year review. J. Pediatr. Surg. 49(2), 280-283 (2014).
-
(2014)
J. Pediatr. Surg.
, vol.49
, Issue.2
, pp. 280-283
-
-
Farrelly, P.J.1
Charlesworth, C.2
Lee, S.3
Southern, K.W.4
Baillie, C.T.5
-
3
-
-
43249109661
-
A single centre experience of liver disease in adults with cystic fibrosis 1995-2006
-
Nash KL, Allison ME, McKeon D, Lomas DJ, Haworth CS, Bilton D, Alexander GJ. A single centre experience of liver disease in adults with cystic fibrosis 1995-2006. J. Cyst. Fibros. 7(3), 252-257 (2007).
-
(2007)
J. Cyst. Fibros.
, vol.7
, Issue.3
, pp. 252-257
-
-
Nash, K.L.1
Allison, M.E.2
McKeon, D.3
Lomas, D.J.4
Haworth, C.S.5
Bilton, D.6
Alexander, G.J.7
-
5
-
-
84884366610
-
Bacterial sinusitis can be a focus for initial lung colonisation and chronic lung infection in patients with cystic fibrosis
-
Aanæs K. Bacterial sinusitis can be a focus for initial lung colonisation and chronic lung infection in patients with cystic fibrosis. J. Cyst. Firbros. 12(Suppl. 2), S1-S20 (2013).
-
(2013)
J. Cyst. Firbros.
, vol.12
, Issue.SUPPL. 2
-
-
Aanæs, K.1
-
6
-
-
77955292433
-
Airway microbiota and pathogen abundance in age-stratified cystic fibrosis patients
-
Cox MJ, Allgaier M, Taylor B et al. Airway microbiota and pathogen abundance in age-stratified cystic fibrosis patients. PLoS ONE 5(6), e11044 (2010).
-
(2010)
PLoS ONE
, vol.5
, Issue.6
-
-
Cox, M.J.1
Allgaier, M.2
Taylor, B.3
-
7
-
-
84880382304
-
Staphylococcus aureus small-colony variants are independently associated with worse lung disease in children with cystic fibrosis
-
Wolter DJ, Emerson JC, McNamara S et al. Staphylococcus aureus small-colony variants are independently associated with worse lung disease in children with cystic fibrosis. Clin. Infect. Dis. 57(3), 384-391 (2013).
-
(2013)
Clin. Infect. Dis.
, vol.57
, Issue.3
, pp. 384-391
-
-
Wolter, D.J.1
Emerson, J.C.2
McNamara, S.3
-
8
-
-
84898874709
-
Biogeochemical forces shape the composition and physiology of polymicrobial communities in the cystic fibrosis lung
-
Quinn RA, Lim YW, Maughan H, Conrad D, Rohwer F, Whiteson KL. Biogeochemical forces shape the composition and physiology of polymicrobial communities in the cystic fibrosis lung. MBio 5(2), e00956-e01013 (2014).
-
(2014)
MBio
, vol.5
, Issue.2
-
-
Quinn, R.A.1
Lim, Y.W.2
Maughan, H.3
Conrad, D.4
Rohwer, F.5
Whiteson, K.L.6
-
9
-
-
0023205299
-
Chest physiotherapy-the mechanical approach to antiinfective therapy in cystic fibrosis
-
Zach MS, Oberwaldner B. Chest physiotherapy-the mechanical approach to antiinfective therapy in cystic fibrosis. Infection 15(5), 381-384 (1987).
-
(1987)
Infection
, vol.15
, Issue.5
, pp. 381-384
-
-
Zach, M.S.1
Oberwaldner, B.2
-
10
-
-
18944382506
-
Conventional chest physiotherapy compared with other airway clearance techniques in cystic fibrosis
-
Main E, Prasad A, Schans C. Conventional chest physiotherapy compared with other airway clearance techniques in cystic fibrosis. Cochrane Database Syst. Rev. 1, CD002011 (2005).
-
(2005)
Cochrane Database Syst. Rev.
, vol.1
-
-
Main, E.1
Prasad, A.2
Schans, C.3
-
12
-
-
0025181636
-
The origin of DNA associated with mucus glycoproteins in cystic fibrosis sputum
-
Letham MI, James SL, Marriott C, Burke JF. The origin of DNA associated with mucus glycoproteins in cystic fibrosis sputum. Eur. Respir. J. 3(1), 19-23 (1990).
-
(1990)
Eur. Respir. J.
, vol.3
, Issue.1
, pp. 19-23
-
-
Letham, M.I.1
James, S.L.2
Marriott, C.3
Burke, J.F.4
-
13
-
-
66849097677
-
Mucus, phlegm, and sputum in cystic fibrosis
-
Rubin B. Mucus, phlegm, and sputum in cystic fibrosis. Respir. Care 54(6), 726-732 (2009).
-
(2009)
Respir. Care
, vol.54
, Issue.6
, pp. 726-732
-
-
Rubin, B.1
-
14
-
-
22544462496
-
Nonmucoid Pseudomonas aeruginosa expresses alginate in the lungs of patients with cystic fibrosis and in a mouse model
-
Bragonzi A, Worlitzsch D, Pier GB, Timpert P, Ulrich M, Hentzer M, Andersen JB, Givskov M, Conese M. Nonmucoid Pseudomonas aeruginosa expresses alginate in the lungs of patients with cystic fibrosis and in a mouse model. J. Infect. Dis. 192(3), 410-419 (2005).
-
(2005)
J. Infect. Dis.
, vol.192
, Issue.3
, pp. 410-419
-
-
Bragonzi, A.1
Worlitzsch, D.2
Pier, G.B.3
Timpert, P.4
Ulrich, M.5
Hentzer, M.6
Andersen, J.B.7
Givskov, M.8
Conese, M.9
-
15
-
-
80054690786
-
Bacteriophages and diffusion of genes encoding antimicrobial resistance in cystic fibrosis sputum microbiota
-
Fancello L, Desnues C, Raoult D, Rolain JM. Bacteriophages and diffusion of genes encoding antimicrobial resistance in cystic fibrosis sputum microbiota. J. Antimicrob. Chemother. 66(11), 2448-2454 (2011).
-
(2011)
J. Antimicrob. Chemother.
, vol.66
, Issue.11
, pp. 2448-2454
-
-
Fancello, L.1
Desnues, C.2
Raoult, D.3
Rolain, J.M.4
-
16
-
-
77950648700
-
Genetic adaptation of Pseudomonas aeruginosa during chronic lung infection of patients with cystic fibrosis: Strong and weak mutators with heterogeneous genetic backgrounds emerge in mucA and/or lasR mutants
-
Ciofu O, Mandsberg LF, Bjarnsholt T, Wassermann T, H∅iby N. Genetic adaptation of Pseudomonas aeruginosa during chronic lung infection of patients with cystic fibrosis: strong and weak mutators with heterogeneous genetic backgrounds emerge in mucA and/or lasR mutants. Microbiology 156(Pt 4), 1108-1119 (2010).
-
(2010)
Microbiology
, vol.156
, Issue.PART 4
, pp. 1108-1119
-
-
Ciofu, O.1
Mandsberg, L.F.2
Bjarnsholt, T.3
Wassermann, T.4
Heiby, N.5
-
17
-
-
79957586352
-
Role of persister cells in chronic infections: Clinical relevance and perspectives on anti-persister therapies
-
Fauvart M1, De Groote VN, Michiels J. Role of persister cells in chronic infections: clinical relevance and perspectives on anti-persister therapies. J. Med. Microbiol. 60(6), 699-709 (2011).
-
(2011)
J. Med. Microbiol.
, vol.60
, Issue.6
, pp. 699-709
-
-
Fauvart, M.1
De Groote, V.N.2
Michiels, J.3
-
18
-
-
77957947243
-
Emergence of Pseudomonas aeruginosa strains producing high levels of persister cells in patients with cystic fibrosis
-
Mulcahy LR1, Burns JL, Lory S, Lewis K. Emergence of Pseudomonas aeruginosa strains producing high levels of persister cells in patients with cystic fibrosis. J. Bacteriol. 192(23), 6191-6199 (2010).
-
(2010)
J. Bacteriol.
, vol.192
, Issue.23
, pp. 6191-6199
-
-
Lr, M.1
Burns, J.L.2
Lory, S.3
Lewis, K.4
-
19
-
-
33846314338
-
A novel host defense system of airways is defective in cystic fibrosis
-
Moskwa P, Lorentzen D, Excoffon KJ et al. A novel host defense system of airways is defective in cystic fibrosis. Am. J. Respir. Crit. Care Med. 175(2), 174-183 (2007).
-
(2007)
Am. J. Respir. Crit. Care Med.
, vol.175
, Issue.2
, pp. 174-183
-
-
Moskwa, P.1
Lorentzen, D.2
Excoffon, K.J.3
-
20
-
-
3242702434
-
Impaired nitric oxide synthase-2 signaling pathway in cystic fibrosis airway epithelium
-
Zheng S, Xu W, Bose S, Banerjee AK, Haque SJ, Erzurum SC. Impaired nitric oxide synthase-2 signaling pathway in cystic fibrosis airway epithelium. Am. J. Physiol. Lung Cell. Mol. Physiol. 287(2), L374-L381 (2004).
-
(2004)
Am. J. Physiol. Lung Cell. Mol. Physiol.
, vol.287
, Issue.2
-
-
Zheng, S.1
Xu, W.2
Bose, S.3
Banerjee, A.K.4
Haque, S.J.5
Erzurum, S.C.6
-
21
-
-
77956396747
-
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition
-
Luciani A, Villella VR, Esposito S et al. Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition. Nat. Cell. Biol. 12(9), 863-875 (2010).
-
(2010)
Nat. Cell. Biol.
, vol.12
, Issue.9
, pp. 863-875
-
-
Luciani, A.1
Villella, V.R.2
Esposito, S.3
-
22
-
-
33847711906
-
Cystic fibrosis mortality and survival in the UK: 1947-2003
-
Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur. Resp. J. 29, 522-526 (2007).
-
(2007)
Eur. Resp. J.
, vol.29
, pp. 522-526
-
-
Dodge, J.A.1
Lewis, P.A.2
Stanton, M.3
Wilsher, J.4
-
23
-
-
84867981708
-
Antimicrobial susceptibility and synergy studies of cystic fibrosis sputum by direct sputum sensitivity testing
-
Serisier DJ, Tuck A, Matley D, Carroll MP, Jones G. Antimicrobial susceptibility and synergy studies of cystic fibrosis sputum by direct sputum sensitivity testing. Eur. J. Clin. Microbiol. Infect. Dis. 31(11), 3211-3216.
-
Eur. J. Clin. Microbiol. Infect. Dis.
, vol.31
, Issue.11
, pp. 3211-3216
-
-
Serisier, D.J.1
Tuck, A.2
Matley, D.3
Carroll, M.P.4
Jones, G.5
-
24
-
-
77955302040
-
Side effects of aminoglycosides on the kidney, ear and balance in cystic fibrosis
-
Prayle A, Watson A, Fortnum H, Smyth A. Side effects of aminoglycosides on the kidney, ear and balance in cystic fibrosis. Thorax 65(7), 654-658 (2010).
-
(2010)
Thorax
, vol.65
, Issue.7
, pp. 654-658
-
-
Prayle, A.1
Watson, A.2
Fortnum, H.3
Smyth, A.4
-
25
-
-
0038164802
-
Pulmonary transplantation for cystic fibrosis: Pre-transplant recipient characteristics in patients dying of peri-operative sepsis
-
De Soyza A, Archer L, Wardle J, Parry G, Dark JH, Gould K, Corris PA. Pulmonary transplantation for cystic fibrosis: pre-transplant recipient characteristics in patients dying of peri-operative sepsis. J. Heart Lung Transplant. 22(7), 764-769 (2003).
-
(2003)
J. Heart Lung Transplant.
, vol.22
, Issue.7
, pp. 764-769
-
-
De Soyza, A.1
Archer, L.2
Wardle, J.3
Parry, G.4
Dark, J.H.5
Gould, K.6
Corris, P.A.7
-
26
-
-
0036220978
-
Antibiotic prophylaxis in infants and young children with cystic fibrosis: A randomized controlled trial
-
Stutman HR, Lieberman JM, Nussbaum E, Marks MI. Antibiotic prophylaxis in infants and young children with cystic fibrosis: a randomized controlled trial. J. Pediatr. 140(3), 299-305 (2002).
-
(2002)
J. Pediatr.
, vol.140
, Issue.3
, pp. 299-305
-
-
Stutman, H.R.1
Lieberman, J.M.2
Nussbaum, E.3
Marks, M.I.4
-
27
-
-
79953284775
-
Inhaled antibiotics for long-term therapy in cystic fibrosis
-
Ryan G, Singh M, Dwan K. Inhaled antibiotics for long-term therapy in cystic fibrosis. Cochrane Database Syst Rev. 3, CD001021 (2011).
-
(2011)
Cochrane Database Syst Rev.
, vol.3
-
-
Ryan, G.1
Singh, M.2
Dwan, K.3
-
28
-
-
84879411129
-
Oral, inhaled, and intravenous antibiotic choice for treating pulmonary exacerbations in cystic fibrosis
-
Wagener JS, Rasouliyan L, VanDevanter DR et al. Oral, inhaled, and intravenous antibiotic choice for treating pulmonary exacerbations in cystic fibrosis. Pediatr. Pulmonol. 48(7), 666-673 (2013).
-
(2013)
Pediatr. Pulmonol.
, vol.48
, Issue.7
, pp. 666-673
-
-
Wagener, J.S.1
Rasouliyan, L.2
Vandevanter, D.R.3
-
29
-
-
0030573808
-
Spread of beta-lactam-resistant Pseudomonas aeruginosa in a cystic fibrosis clinic
-
Cheng K, Smyth RL, Govan JR et al. Spread of beta-lactam-resistant Pseudomonas aeruginosa in a cystic fibrosis clinic. Lancet. 348(9028),639-642 (1996).
-
(1996)
Lancet
, vol.348
, Issue.9028
, pp. 639-642
-
-
Cheng, K.1
Smyth, R.L.2
Govan, J.R.3
-
30
-
-
0018099851
-
Acetaminophen poisoning: A case report of the use of acetylcysteine
-
Scalley RD, Conner CS. Acetaminophen poisoning: a case report of the use of acetylcysteine. Am. J. Hosp. Pharm. 35(8), 964-967 (1978).
-
(1978)
Am. J. Hosp. Pharm.
, vol.35
, Issue.8
, pp. 964-967
-
-
Scalley, R.D.1
Conner, C.S.2
-
31
-
-
84880170145
-
Inhalation treatment with glutathione in patients with cystic fibrosis. A randomized clinical trial
-
Griese M, Kappler M, Eismann C et al. Inhalation treatment with glutathione in patients with cystic fibrosis. A randomized clinical trial. Am. J. Respir. Crit. Care Med. 188(1), 83-89 (2013).
-
(2013)
Am. J. Respir. Crit. Care Med.
, vol.188
, Issue.1
, pp. 83-89
-
-
Griese, M.1
Kappler, M.2
Eismann, C.3
-
32
-
-
84891638906
-
Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis
-
Tam J, Nash EF, Ratjen F, Tullis E, Stephenson A. Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis. Cochrane Database Syst. Rev. 7, CD007168 (2013).
-
(2013)
Cochrane Database Syst. Rev.
, vol.7
-
-
Tam, J.1
Nash, E.F.2
Ratjen, F.3
Tullis, E.4
Stephenson, A.5
-
33
-
-
84878935910
-
Adherence to dornase alfa treatment among commercially insured patients with cystic fibrosis
-
Nasr SZ, Chou W, Villa KF, Chang E, Broder MS. Adherence to dornase alfa treatment among commercially insured patients with cystic fibrosis. J. Med. Econ. 16(6), 801-808 (2013).
-
(2013)
J. Med. Econ.
, vol.16
, Issue.6
, pp. 801-808
-
-
Nasr, S.Z.1
Chou, W.2
Villa, K.F.3
Chang, E.4
Broder, M.S.5
-
34
-
-
0030024897
-
Dornase alfa: A new option in the management of cystic fibrosis
-
Witt DM, Anderson L. Dornase alfa: a new option in the management of cystic fibrosis. Pharmacotherapy 16(1), 40-48 (1996)
-
(1996)
Pharmacotherapy
, vol.16
, Issue.1
, pp. 40-48
-
-
Witt, D.M.1
Anderson, L.2
-
36
-
-
84895819826
-
-
US FDA NDA# 202049: mannitol inhalation powder (proposed trade name Bronchitol) for oral inhalation sponsored by Pharmaxis, for the management of cystic fibrosis (CF) in patients aged 6 years and older to improve pulmonary function.
-
US FDA. Pulmonary-allergy drugs advisory committee meeting (2013).NDA# 202049: mannitol inhalation powder (proposed trade name Bronchitol) for oral inhalation sponsored by Pharmaxis, for the management of cystic fibrosis (CF) in patients aged 6 years and older to improve pulmonary function. www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/ Pulmonary-AllergyDrugsAdvisoryCommittee/UCM336995.pdf
-
(2013)
Pulmonary-allergy Drugs Advisory Committee Meeting
-
-
-
37
-
-
84879245725
-
-
European Medicines AgencyCommittee for Medicinal Products for Human Use
-
European Medicines Agency, Committee for Medicinal Products for Human Use (2012). CHMP Assessment Report, Bronchitol® . www.ema.europa.eu/docs/en-GB/ document-library/EPAR-Public-assessment-report/human/001252/WC500130591.pdf
-
(2012)
CHMP Assessment Report, Bronchitol®.
-
-
-
38
-
-
84997743625
-
-
National Institute for Health and Clinical Excellence
-
National Institute for Health and Clinical Excellence (2012). Mannitol dry powder for inhalation for treating cystic fibrosis [online]. www.nice.org.uk/nicemedia/live/13969/61603/61603.pdf
-
(2012)
Mannitol Dry Powder for Inhalation for Treating Cystic Fibrosis
-
-
-
39
-
-
84905193952
-
-
Scottish Medicines Consortium
-
Scottish Medicines Consortium (2013). Mannitol, 400 mg, inhalation powder, hard capsule, (Bronchitol® ). www.scottishmedicines.org.uk/files/ advice/inhaled-mannitol-Bronchitol-FINAL-January-2013-for-website. pdf
-
(2013)
Mannitol, 400 Mg, Inhalation Powder, Hard Capsule, (Bronchitol® )
-
-
-
40
-
-
84880078608
-
A Phase III randomised study of the efficacy and safety of inhaled dry powder mannitol (Bronchitol) for the symptomatic treatment of non-cystic fibrosis bronchiectasis
-
Bilton D, Daviskas E, Anderson SD et al. A Phase III randomised study of the efficacy and safety of inhaled dry powder mannitol (Bronchitol) for the symptomatic treatment of non-cystic fibrosis bronchiectasis. Chest 144(1), 215-225 (2013).
-
(2013)
Chest
, vol.144
, Issue.1
, pp. 215-225
-
-
Bilton, D.1
Daviskas, E.2
Anderson, S.D.3
-
41
-
-
84892500332
-
Mannitol enhances antibiotic sensitivity of persister bacteria in Pseudomonas aeruginosa biofilms
-
Barraud N, Buson A, Jarolimek W, Rice SA. Mannitol enhances antibiotic sensitivity of persister bacteria in Pseudomonas aeruginosa biofilms. PLoS ONE 8(12), e84220 (2013).
-
(2013)
PLoS ONE
, vol.8
, Issue.12
-
-
Barraud, N.1
Buson, A.2
Jarolimek, W.3
Rice, S.A.4
-
42
-
-
0034835277
-
Microbiological and immunologic considerations with aerosolized drug delivery
-
LiPuma JJ. Microbiological and immunologic considerations with aerosolized drug delivery. Chest 120, 18S-23S (2001).
-
(2001)
Chest
, vol.120
-
-
Lipuma, J.J.1
-
44
-
-
66849121732
-
New aerosol delivery devices for cystic fibrosis
-
Kesser KC, Geller DE. New aerosol delivery devices for cystic fibrosis. Resir. Care. 54(6), 754-767 (2009).
-
(2009)
Resir. Care.
, vol.54
, Issue.6
, pp. 754-767
-
-
Kesser, K.C.1
Geller, D.E.2
-
45
-
-
79958257149
-
Accurate assessment of adherence: Self-report and clinician report vs electronic monitoring of nebulisers
-
Daniels T, Goodacre L, Sutton C, Pollard K, Conway S, Peckham D. Accurate assessment of adherence: self-report and clinician report vs electronic monitoring of nebulisers. Chest 140(2), 425-432 (2011).
-
(2011)
Chest
, vol.140
, Issue.2
, pp. 425-432
-
-
Daniels, T.1
Goodacre, L.2
Sutton, C.3
Pollard, K.4
Conway, S.5
Peckham, D.6
-
46
-
-
79961039597
-
Development of an inhaled dry-powder formulation of tobramycin using PulmoSphere™ technology
-
Geller DE, Weers J, Heuerding S. Development of an inhaled dry-powder formulation of tobramycin using PulmoSphere™ technology. J. Aerosol. Med. Pulm. Drug Deliv. 24(4), 175-182 (2011).
-
(2011)
J. Aerosol. Med. Pulm. Drug Deliv.
, vol.24
, Issue.4
, pp. 175-182
-
-
Geller, D.E.1
Weers, J.2
Heuerding, S.3
-
47
-
-
84888992940
-
Tobramycin inhalation powder in cystic fibrosis patients: Response by age group
-
doi: 10.4187/respcare.02264
-
Geller DE, Nasr SZ, Piggott S, He E, Angyalosi G, Higgins M. Tobramycin inhalation powder in cystic fibrosis patients: response by age group. Respir. Care doi: 10.4187/respcare.02264 (2013)
-
(2013)
Respir. Care
-
-
Geller, D.E.1
Nasr, S.Z.2
Piggott, S.3
He, E.4
Angyalosi, G.5
Higgins, M.6
-
48
-
-
79957529529
-
Aspergillus fumigatus colonization in cystic fibrosis: Implications for lung function?
-
de Vrankrijker AM, van der Ent CK, van Berkhout FT et al. Aspergillus fumigatus colonization in cystic fibrosis: implications for lung function? Clin. Microbiol. Infect. 17(9), 1381-1386 (2011).
-
(2011)
Clin. Microbiol. Infect.
, vol.17
, Issue.9
, pp. 1381-1386
-
-
De Vrankrijker, A.M.1
Van Der Ent, C.K.2
Van Berkhout, F.T.3
-
49
-
-
84900545521
-
Physicochemical compatibility and stability of nebulizable drug admixtures containing dornase alfa and tobramycin
-
doi:10.1016/j.pupt.2013.08.003 Epub ahead of print
-
Klemmer A, Krämer I, Kamin W. Physicochemical compatibility and stability of nebulizable drug admixtures containing dornase alfa and tobramycin. Pulm. Pharmacol. Ther. doi:10.1016/j.pupt.2013.08.003 (2013) Epub ahead of print).
-
(2013)
Pulm. Pharmacol. Ther.
-
-
Klemmer, A.1
Krämer, I.2
Kamin, W.3
-
50
-
-
0038363377
-
A mutation in the cystic fibrosis transmembrane conductance regulator generates a novel internalization sequence and enhances endocytic rates
-
Silvis MR, Picciano JA, Bertrand C, Weixel K, Bridges RJ, Bradbury NA. A mutation in the cystic fibrosis transmembrane conductance regulator generates a novel internalization sequence and enhances endocytic rates. J. Biol. Chem. 278(13), 11554-11560 (2003).
-
(2003)
J. Biol. Chem.
, vol.278
, Issue.13
, pp. 11554-11560
-
-
Silvis, M.R.1
Picciano, J.A.2
Bertrand, C.3
Weixel, K.4
Bridges, R.J.5
Bradbury, N.A.6
-
51
-
-
34247588271
-
PTC 124 targets genetic disorders caused by nonsense mutations
-
Welch EM, Barton ER, Zhuo J et al. PTC 124 targets genetic disorders caused by nonsense mutations. Nature 447(7140), 87-91 (2007).
-
(2007)
Nature
, vol.447
, Issue.7140
, pp. 87-91
-
-
Welch, E.M.1
Barton, E.R.2
Zhuo, J.3
-
52
-
-
79958746554
-
Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking and opportunities for mutation-specific treatment
-
Rogan MP, Stoltz DA, Hornick DB. Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking and opportunities for mutation-specific treatment. Chest 139, 1480-1490 (2011).
-
(2011)
Chest
, vol.139
, pp. 1480-1490
-
-
Rogan, M.P.1
Stoltz, D.A.2
Hornick, D.B.3
-
53
-
-
50149098401
-
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: A prospective Phase II trial
-
Kerem E, Hirawat S, Armoni S et al. Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective Phase II trial. Lancet 372(9640), 719-727 (2008).
-
(2008)
Lancet
, vol.372
, Issue.9640
, pp. 719-727
-
-
Kerem, E.1
Hirawat, S.2
Armoni, S.3
-
54
-
-
62549134976
-
Mechanism of PTC124 activity in cell-based luciferase assays of nonsense codon suppression
-
Auld, DS, Thorne N, Maguire WF, Inglese J. Mechanism of PTC124 activity in cell-based luciferase assays of nonsense codon suppression. Proc. Natl Acad. Sci. USA 106(9), 3585-3590 (2009).
-
(2009)
Proc. Natl Acad. Sci. USA
, vol.106
, Issue.9
, pp. 3585-3590
-
-
Auld, D.S.1
Thorne, N.2
Maguire, W.F.3
Inglese, J.4
-
55
-
-
77950430317
-
Molecular basis for the high-affinity binding and stabilization of firefly luciferase by PTC124
-
Auld DS, Lovell S, Thorne N et al. Molecular basis for the high-affinity binding and stabilization of firefly luciferase by PTC124. Proc Natl Acad Sci. USA 107(11), 4878-4883 (2010).
-
(2010)
Proc Natl Acad Sci. USA
, vol.107
, Issue.11
, pp. 4878-4883
-
-
Auld, D.S.1
Lovell, S.2
Thorne, N.3
-
56
-
-
84879391161
-
A lack of premature termination codon read-through
-
McElroy SP, Nomura T, Torrie LS et al. A lack of premature termination codon read-through. PLoS Biol. 11(6), e1001593 (2013).
-
(2013)
PLoS Biol.
, vol.11
, Issue.6
-
-
McElroy, S.P.1
Nomura, T.2
Torrie, L.S.3
-
57
-
-
78349290383
-
Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis
-
Sermet-Gaudelus I, Boeck KD, Casimir GJ et al. Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis. Am. J. Respir. Crit. Care Med. 182(10), 1262-1272 (2010).
-
(2010)
Am. J. Respir. Crit. Care Med.
, vol.182
, Issue.10
, pp. 1262-1272
-
-
Sermet-Gaudelus, I.1
Boeck, K.D.2
Casimir, G.J.3
-
58
-
-
84905173104
-
-
Cystic Fibrosis Foundation Clinical research information sheet [online]
-
Cystic Fibrosis Foundation, Clinical research information sheet (2012). Ataluren (PTC 124) in Cystic Fibrosis [online]. www.cff.org/UploadedFiles/ ClinicalResearchPDF/CR84.pdf
-
(2012)
Ataluren (PTC 124) in Cystic Fibrosis
-
-
-
59
-
-
84883480295
-
Correction of nonsense BMPR2 and SMAD9 mutations by Ataluren in pulmonary arterial hypertension
-
Drake KM, Dunmore BJ, McNelly LN, Morrell NW, Aldred MA. Correction of nonsense BMPR2 and SMAD9 mutations by Ataluren in pulmonary arterial hypertension. Am. J. Respir. Cell. Mol. Biol. 49(3), 403-409 (2013)
-
(2013)
Am. J. Respir. Cell. Mol. Biol.
, vol.49
, Issue.3
, pp. 403-409
-
-
Drake, K.M.1
Dunmore, B.J.2
McNelly, L.N.3
Morrell, N.W.4
Aldred, M.A.5
-
60
-
-
84901040034
-
Muscle dysfunction and structural defects of dystrophin-null sapje mutant zebrafish larvae are rescued by Ataluren treatment
-
Li M, Andersson-Lendahl M, Sejersen T, Arner A. Muscle dysfunction and structural defects of dystrophin-null sapje mutant zebrafish larvae are rescued by Ataluren treatment. FASEB J. 28(4), 1593-1599 (2013).
-
(2013)
FASEB J.
, vol.28
, Issue.4
, pp. 1593-1599
-
-
Li, M.1
Andersson-Lendahl, M.2
Sejersen, T.3
Arner, A.4
-
61
-
-
84892581676
-
Phase 2a study of Ataluren-mediated dystrophin production in patients with nonsense mutation duchenne muscular dystrophy
-
Finkel RS, Flanigan KM, Wong B et al. Phase 2a study of Ataluren-mediated dystrophin production in patients with nonsense mutation duchenne muscular dystrophy. PLoS ONE 8(12), e81302 (2013).
-
(2013)
PLoS ONE
, vol.8
, Issue.12
-
-
Finkel, R.S.1
Flanigan, K.M.2
Wong, B.3
-
62
-
-
79958746554
-
Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking and opportunities for mutation-specific treatment
-
Rogan MP, Stoltz DA, Hornick DB. Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking and opportunities for mutation-specific treatment. Chest 139, 1480-1490 (2011).
-
(2011)
Chest
, vol.139
, pp. 1480-1490
-
-
Rogan, M.P.1
Stoltz, D.A.2
Hornick, D.B.3
-
63
-
-
84905165723
-
Grootenhuis PDRescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
-
1064418825188302009
-
VanGoor F, Hadida S, Grootenhuis PDRescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770Proc. Natl Acad. Sci. USA.1064418825188302009
-
Proc. Natl Acad. Sci. USA.
-
-
Vangoor, F.1
Hadida, S.2
-
64
-
-
84868243529
-
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner
-
Eckford PD, Li C, Ramjeesingh M, Bear CE. Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner. J. Biol. Chem. 287(44), 36639-36649 (2012).
-
(2012)
J. Biol. Chem.
, vol.287
, Issue.44
, pp. 36639-36649
-
-
Eckford, P.D.1
Li, C.2
Ramjeesingh, M.3
Bear, C.E.4
-
65
-
-
84875048537
-
Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle
-
Jih KY, Hwang TC. Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle. Proc. Natl Acad. Sci. USA 110(11), 4404-4409 (2013).
-
(2013)
Proc. Natl Acad. Sci. USA
, vol.110
, Issue.11
, pp. 4404-4409
-
-
Jih, K.Y.1
Hwang, T.C.2
-
66
-
-
80455162465
-
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
-
Ramsey BW, Davies J, McElvaney NG et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N. Engl. J. Med. 365(18), 1663-1672 (2011).
-
(2011)
N. Engl. J. Med.
, vol.365
, Issue.18
, pp. 1663-1672
-
-
Ramsey, B.W.1
Davies, J.2
McElvaney, N.G.3
-
67
-
-
84884773595
-
VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
-
Ren HY, Grove DE, De La Rosa O et al. VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1. Mol. Biol. Cell. 24(19), 3016-3024 (2013).
-
(2013)
Mol. Biol. Cell.
, vol.24
, Issue.19
, pp. 3016-3024
-
-
Ren, H.Y.1
Grove, D.E.2
De La Rosa, O.3
-
68
-
-
84884909640
-
Corrector VX-809 stabilizes the first transmembrane domain of CFTR
-
Loo TW, Bartlett MC, Clarke DM. Corrector VX-809 stabilizes the first transmembrane domain of CFTR. Biochem. Pharmacol. 86(5), 612-619 (2013).
-
(2013)
Biochem. Pharmacol.
, vol.86
, Issue.5
, pp. 612-619
-
-
Loo, T.W.1
Bartlett, M.C.2
Clarke, D.M.3
-
69
-
-
84880894290
-
Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction
-
Farinha CM, King-Underwood J, Sousa M et al. Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction. Chem. Biol. 20(7), 943-955 (2013).
-
(2013)
Chem. Biol.
, vol.20
, Issue.7
, pp. 943-955
-
-
Farinha, C.M.1
King-Underwood, J.2
Sousa, M.3
-
70
-
-
84873436500
-
Correctors of F508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein
-
He L1, Kota P, Aleksandrov AA, Cui L, Jensen T, Dokholyan NV, Riordan JR. Correctors of ?F508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein. FASEB J. 27(2), 536-545 (2013).
-
(2013)
FASEB J.
, vol.27
, Issue.2
, pp. 536-545
-
-
He, L.1
Kota, P.2
Aleksandrov, A.A.3
Cui, L.4
Jensen, T.5
Dokholyan, N.V.6
Riordan, J.R.7
-
71
-
-
84862908028
-
Correction of both NBD1 energetics and domain interface is required to restore ?F508 CFTR folding and function
-
Rabeh WM, Bossard F, Xu H et al. Correction of both NBD1 energetics and domain interface is required to restore ?F508 CFTR folding and function. Cell 148(1-2), 150-163 (2012).
-
(2012)
Cell
, vol.148
, Issue.1-2
, pp. 150-163
-
-
Rabeh, W.M.1
Bossard, F.2
Xu, H.3
-
72
-
-
84879410121
-
Mechanism-based corrector combination restores ?F508-CFTR folding and function
-
Okiyoneda T1, Veit G, Dekkers JF et al. Mechanism-based corrector combination restores ?F508-CFTR folding and function. Nat. Chem. Biol. 9(7), 444-454 (2013).
-
(2013)
Nat. Chem. Biol.
, vol.9
, Issue.7
, pp. 444-454
-
-
Okiyoneda, T.1
Veit, G.2
Dekkers, J.F.3
-
73
-
-
84855202429
-
Results of a Phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
-
Clancy JP, Rowe SM, Accurso FJ et al. Results of a Phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax 67(1), 12-18 (2012).
-
(2012)
Thorax
, vol.67
, Issue.1
, pp. 12-18
-
-
Clancy, J.P.1
Rowe, S.M.2
Accurso, F.J.3
-
74
-
-
84885670457
-
Managing the underlying cause of cystic fibrosis: A future role for potentiators and correctors
-
Galietta LJ. Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors. Pediatr. Drugs. 15(5), 393-402 (2013).
-
(2013)
Pediatr. Drugs.
, vol.15
, Issue.5
, pp. 393-402
-
-
Galietta, L.J.1
-
75
-
-
84880894290
-
Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction
-
Farinha CM, King-Underwood J, Sousa M et al. Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction. Chem. Biol. 20(7), 943-955 (2013).
-
(2013)
Chem. Biol.
, vol.20
, Issue.7
, pp. 943-955
-
-
Farinha, C.M.1
King-Underwood, J.2
Sousa, M.3
-
76
-
-
0032252622
-
Effect of inhibition of nitric oxide synthase on Pseudomonas aeruginosa infection of respiratory mucosa in vitro
-
Dowling RB, Newton R, Robichaud A, Cole PJ, Barnes PJ, Wilson R. Effect of inhibition of nitric oxide synthase on Pseudomonas aeruginosa infection of respiratory mucosa in vitro. Am. J. Respir. Cell. Mol. Biol. 19(6), 950-958 (1998).
-
(1998)
Am. J. Respir. Cell. Mol. Biol.
, vol.19
, Issue.6
, pp. 950-958
-
-
Dowling, R.B.1
Newton, R.2
Robichaud, A.3
Cole, P.J.4
Barnes, P.J.5
Wilson, R.6
-
77
-
-
0034773245
-
Dexamethasone impairs pulmonary defence against Pseudomonas aeruginosa through suppressing iNOS gene expression and peroxynitrite production in mice
-
Satoh S, Oishi K, Iwagaki A et al. Dexamethasone impairs pulmonary defence against Pseudomonas aeruginosa through suppressing iNOS gene expression and peroxynitrite production in mice. Clin. Exp. Immunol. 126(2), 266-273 (2001).
-
(2001)
Clin. Exp. Immunol.
, vol.126
, Issue.2
, pp. 266-273
-
-
Satoh, S.1
Oishi, K.2
Iwagaki, A.3
-
78
-
-
0027937417
-
Evidence for increased oxidative damage in patients with cystic fibrosis
-
Brown RK, Kelly FJ. Evidence for increased oxidative damage in patients with cystic fibrosis. Pediatr. Res. 36(4), 487-493 (1994).
-
(1994)
Pediatr. Res.
, vol.36
, Issue.4
, pp. 487-493
-
-
Brown, R.K.1
Kelly, F.J.2
-
79
-
-
0030042127
-
Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress
-
Brown RK, Wyatt H, Price JF, Kelly FJ. Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress. Eur. Respir. J. 9(2), 334-339 (1996).
-
(1996)
Eur. Respir. J.
, vol.9
, Issue.2
, pp. 334-339
-
-
Brown, R.K.1
Wyatt, H.2
Price, J.F.3
Kelly, F.J.4
-
80
-
-
84893348472
-
Reduced nasal nitric oxide production in cystic fibrosis patients with elevated systemic inflammation markers
-
Michl RK, Hentschel J, Fischer C, Beck JF, Mainz JG. Reduced nasal nitric oxide production in cystic fibrosis patients with elevated systemic inflammation markers. PLoS ONE 8(11), e79141 (2013).
-
(2013)
PLoS ONE
, vol.8
, Issue.11
-
-
Michl, R.K.1
Hentschel, J.2
Fischer, C.3
Beck, J.F.4
Mainz, J.G.5
-
81
-
-
84874712290
-
Upper and lower airway nitric oxide levels in primary ciliary dyskinesia, cystic fibrosis and asthma
-
Walker WT, Liew A, Harris A, Cole J, Lucas JS. Upper and lower airway nitric oxide levels in primary ciliary dyskinesia, cystic fibrosis and asthma. Respir. Med. 107(3), 380-386 (2013).
-
(2013)
Respir. Med.
, vol.107
, Issue.3
, pp. 380-386
-
-
Walker, W.T.1
Liew, A.2
Harris, A.3
Cole, J.4
Lucas, J.S.5
-
82
-
-
30444439420
-
Increased arginase activity in cystic fibrosis airways
-
Grasemann H, Schwiertz R, Matthiesen S, Racké K, Ratjen F. Increased arginase activity in cystic fibrosis airways. Am. J. Respir. Crit. Care Med. 172(12), 1523-1528 (2005).
-
(2005)
Am. J. Respir. Crit. Care Med.
, vol.172
, Issue.12
, pp. 1523-1528
-
-
Grasemann, H.1
Schwiertz, R.2
Matthiesen, S.3
Racké, K.4
Ratjen, F.5
-
83
-
-
33745739549
-
Decreased systemic bioavailability of L-arginine in patients with cystic fibrosis
-
Grasemann H, Schwiertz R, Grasemann C, Vester U, Racké K, Ratjen F. Decreased systemic bioavailability of L-arginine in patients with cystic fibrosis. Respir. Res. 7, 87 (2006).
-
(2006)
Respir. Res.
, vol.7
, pp. 87
-
-
Grasemann, H.1
Schwiertz, R.2
Grasemann, C.3
Vester, U.4
Racké, K.5
Ratjen, F.6
-
84
-
-
14844317633
-
A pilot study of oral L-arginine in cystic fibrosis
-
Everard ML, Donnelly D. A pilot study of oral L-arginine in cystic fibrosis. J. Cyst. Fibros. 4(1), 67-69 (2005).
-
(2005)
J. Cyst. Fibros.
, vol.4
, Issue.1
, pp. 67-69
-
-
Everard, M.L.1
Donnelly, D.2
-
85
-
-
13444292529
-
Oral L-arginine supplementation in cystic fibrosis patients: A placebo-controlled study
-
Grasemann H, Grasemann C, Kurtz F, Tietze-Schillings G, Vester U, Ratjen F. Oral L-arginine supplementation in cystic fibrosis patients: a placebo-controlled study. Eur. Respir. J. 25(1), 62-68 (2005).
-
(2005)
Eur. Respir. J.
, vol.25
, Issue.1
, pp. 62-68
-
-
Grasemann, H.1
Grasemann, C.2
Kurtz, F.3
Tietze-Schillings, G.4
Vester, U.5
Ratjen, F.6
-
86
-
-
84882882270
-
A randomized controlled trial of inhaled L-arginine in patients with cystic fibrosis
-
Grasemann H, Tullis E, Ratjen F. A randomized controlled trial of inhaled L-arginine in patients with cystic fibrosis. J. Cyst. Fibros. 12(5), 468-474 (2013).
-
(2013)
J. Cyst. Fibros.
, vol.12
, Issue.5
, pp. 468-474
-
-
Grasemann, H.1
Tullis, E.2
Ratjen, F.3
-
87
-
-
84888024804
-
Inhaled nitric oxide decreases the bacterial load in a rat model of Pseudomonas aeruginosa pneumonia
-
Miller CC, Hergott CA, Rohan M, Arsenault-Mehta K, Döring G, Mehta S. Inhaled nitric oxide decreases the bacterial load in a rat model of Pseudomonas aeruginosa pneumonia. J. Cyst. Fibros. 12(6), 817-820 (2013).
-
(2013)
J. Cyst. Fibros.
, vol.12
, Issue.6
, pp. 817-820
-
-
Miller, C.C.1
Hergott, C.A.2
Rohan, M.3
Arsenault-Mehta, K.4
Döring, G.5
Mehta, S.6
-
88
-
-
33744461829
-
Anti-inflammatory effect of augmented nitric oxide production in chronic lung infection
-
Hopkins N, Gunning Y, O'Croinin DF, Laffey JG, McLoughlin P. Anti-inflammatory effect of augmented nitric oxide production in chronic lung infection. J. Pathol. 209(2), 198-205 (2006).
-
(2006)
J. Pathol.
, vol.209
, Issue.2
, pp. 198-205
-
-
Hopkins, N.1
Gunning, Y.2
O'Croinin, D.F.3
Laffey, J.G.4
McLoughlin, P.5
-
89
-
-
84879015771
-
Nebulized thiocyanate improves lung infection outcomes in mice
-
Chandler JD, Min E, Huang J, Nichols DP, Day BJ. Nebulized thiocyanate improves lung infection outcomes in mice. Br. J. Pharmacol. 169(5), 1166-1177 (2013).
-
(2013)
Br. J. Pharmacol.
, vol.169
, Issue.5
, pp. 1166-1177
-
-
Chandler, J.D.1
Min, E.2
Huang, J.3
Nichols, D.P.4
Day, B.J.5
-
90
-
-
57649203344
-
Autophagy in aging, disease and death: The true identity of a cell death impostor
-
Levine B, Kroemer G. Autophagy in aging, disease and death: the true identity of a cell death impostor. Cell Death Differ. 16(1), 1-2 (2009).
-
(2009)
Cell Death Differ.
, vol.16
, Issue.1
, pp. 1-2
-
-
Levine, B.1
Kroemer, G.2
-
91
-
-
84883174355
-
Autophagy enhances bacterial clearance during P. Aeruginosa lung infection
-
Junkins RD, Shen A, Rosen K, McCormick C, Lin TJ. Autophagy enhances bacterial clearance during P. aeruginosa lung infection. PLoS ONE 8(8), e72263 (2013).
-
(2013)
PLoS ONE
, vol.8
, Issue.8
-
-
Junkins, R.D.1
Shen, A.2
Rosen, K.3
McCormick, C.4
Lin, T.J.5
-
92
-
-
0025155528
-
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
-
Rich DP, Anderson MP, Gregory RJ et al. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 347(6291), 358-363 (1990).
-
(1990)
Nature
, vol.347
, Issue.6291
, pp. 358-363
-
-
Rich, D.P.1
Anderson, M.P.2
Gregory, R.J.3
-
93
-
-
0028387979
-
Correction of the apical membrane chloride permeability defect in polarized cystic fibrosis airway epithelia following retroviral-mediated gene transfer
-
Caplen NJ, Gao X, Hayes P et al. Gene therapy for cystic fibrosis in humans by liposome-mediated DNA transfer: the production of resources and the regulatory process. Gene Ther. 1(2), 139-147 (1994).
-
(1994)
Gen Ther
, vol.1
, Issue.2
, pp. 139-147
-
-
Olsen, J.C.1
Johnson, L.G.2
Stutts, M.J.3
Sarkadi, B.4
Yankaskas, J.R.5
Swanstrom, R.6
Boucher, R.C.7
-
94
-
-
0028870361
-
Gene therapy for cystic fibrosis
-
Johnson LG. Gene therapy for cystic fibrosis. Chest 107(2 Suppl.), 77S-83S (1995).
-
(1995)
Chest
, vol.107
, Issue.2 SUPPL.
-
-
Johnson, L.G.1
-
95
-
-
0033533669
-
Virus treatment questioned after gene therapy death
-
Lehrman S. Virus treatment questioned after gene therapy death. Nature 401(6753), 517-518 (1999).
-
(1999)
Nature
, vol.401
, Issue.6753
, pp. 517-518
-
-
Lehrman, S.1
-
96
-
-
84877593162
-
Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation
-
Horsley AR, Davies JC, Gray RD, Macleod KA, Donovan J, Aziz ZA, Bell NJ, Rainer M, Mt-Isa S, Voase N, Dewar MH, Saunders C, Gibson JS, Parra-Leiton J, Larsen MD, Jeswiet S, Soussi S, Bakar Y, Meister MG, Tyler P, Doherty A, Hansell DM, Ashby D, Hyde SC, Gill DR, Greening AP, Porteous DJ, Innes JA, Boyd AC, Griesenbach U, Cunningham S, Alton EW. Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation. Thorax 68(6), 532-539 (2013).
-
(2013)
Thorax
, vol.68
, Issue.6
, pp. 532-539
-
-
Horsley, A.R.1
Davies, J.C.2
Gray, R.D.3
MacLeod, K.A.4
Donovan, J.5
Aziz, Z.A.6
Bell, N.J.7
Rainer, M.8
Mt-Isa, S.9
Voase, N.10
Dewar, M.H.11
Saunders, C.12
Gibson, J.S.13
Parra-Leiton, J.14
Larsen, M.D.15
Jeswiet, S.16
Soussi, S.17
Bakar, Y.18
Meister, M.G.19
Tyler, P.20
Doherty, A.21
Hansell, D.M.22
Ashby, D.23
Hyde, S.C.24
Gill, D.R.25
Greening, A.P.26
Porteous, D.J.27
Innes, J.A.28
Boyd, A.C.29
Griesenbach, U.30
Cunningham, S.31
Alton, E.W.32
more..
-
97
-
-
84895069609
-
Lung clearance index: Evidence for use in clinical trials in cystic fibrosis
-
Kent L, Reix P, Innes JA et al. Lung clearance index: evidence for use in clinical trials in cystic fibrosis. J. Cyst. Fibros. 13(2), 123-138 (2014).
-
(2014)
J. Cyst. Fibros.
, vol.13
, Issue.2
, pp. 123-138
-
-
Kent, L.1
Reix, P.2
Innes, J.A.3
-
98
-
-
84885869516
-
A randomised, double-blind, placebo-controlled Phase IIb clinical trial of repeated application of gene therapy in patients with cystic fibrosis
-
Alton EW, Boyd AC, Cheng SH et al. A randomised, double-blind, placebo-controlled Phase IIb clinical trial of repeated application of gene therapy in patients with cystic fibrosis. Thorax 68(11), 1075-1077 (2013).
-
(2013)
Thorax
, vol.68
, Issue.10
, pp. 1075-1077
-
-
Alton, E.W.1
Boyd, A.C.2
Cheng, S.H.3
-
99
-
-
84896957356
-
The emerging potential of autophagy-based therapies in the treatment of cystic fibrosis lung infections
-
Junkins RD, McCormick C, Lin TJ. The emerging potential of autophagy-based therapies in the treatment of cystic fibrosis lung infections. Autophagy 10(3), 538-547 (2014).
-
(2014)
Autophagy
, vol.10
, Issue.3
, pp. 538-547
-
-
Junkins, R.D.1
McCormick, C.2
Lin, T.J.3
-
100
-
-
84900788535
-
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disdisease
-
Lee TW, Southern KW. Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disdisease. Cochrane Database Syst Rev. 11, CD005599 (2013).
-
(2013)
Cochrane Database Syst Rev.
, vol.11
-
-
Lee, T.W.1
Southern, K.W.2
|