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Simultaneous sulfur hexafluoride and nitrogen multiple-breath washout (MBW) to examine inherent differences in MBW outcomes.

Bayfield KJ, Horsley A, Alton E, Irving S, Bush A, Davies JC.

ERJ Open Res. 2019 Nov 4;5(4). pii: 00234-2018. doi: 10.1183/23120541.00234-2018. eCollection 2019 Oct.


Training dogs to differentiate Pseudomonas aeruginosa from other cystic fibrosis bacterial pathogens: not to be sniffed at?

Davies JC, Alton E, Simbo A, Murphy R, Seth I, Williams K, Somerville M, Jolly L, Morant S, Guest C.

Eur Respir J. 2019 Nov 7;54(5). pii: 1900970. doi: 10.1183/13993003.00970-2019. Print 2019 Nov. No abstract available.


Abnormal pro-gly-pro pathway and airway neutrophilia in pediatric cystic fibrosis.

Turnbull AR, Pyle CJ, Patel DF, Jackson PL, Hilliard TN, Regamey N, Tan HL, Brown S, Thursfield R, Short C, Mc Fie M, Alton EWFW, Gaggar A, Blalock JE, Lloyd CM, Bush A, Davies JC, Snelgrove RJ.

J Cyst Fibros. 2019 Jun 5. pii: S1569-1993(19)30769-6. doi: 10.1016/j.jcf.2019.05.017. [Epub ahead of print]


Metabolic Phenotyping and Strain Characterisation of Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients Using Rapid Evaporative Ionisation Mass Spectrometry.

Bardin EE, Cameron SJS, Perdones-Montero A, Hardiman K, Bolt F, Alton EWFW, Bush A, Davies JC, Takáts Z.

Sci Rep. 2018 Jul 19;8(1):10952. doi: 10.1038/s41598-018-28665-7.


The murine lung as a factory to produce secreted intrapulmonary and circulatory proteins.

Paul-Smith MC, Pytel KM, Gelinas JF, McIntosh J, Pringle I, Davies L, Chan M, Meng C, Bell R, Cammack L, Moran C, Cameron L, Inoue M, Tsugumine S, Hironaka T, Gill DR, Hyde SC, Nathwani A, Alton EWFW, Griesenbach U.

Gene Ther. 2018 Aug;25(5):345-358. doi: 10.1038/s41434-018-0025-8. Epub 2018 Jul 18.


Children with cystic fibrosis demonstrate no respiratory immunological, infective or physiological, consequences of vitamin D deficiency.

Thursfield RM, Naderi K, Leaver N, Rosenthal M, Alton EWFW, Bush A, Davies JC.

J Cyst Fibros. 2018 Sep;17(5):657-665. doi: 10.1016/j.jcf.2018.02.011. Epub 2018 Apr 7.


Impact of T2R38 Receptor Polymorphisms on Pseudomonas aeruginosa Infection in Cystic Fibrosis.

Turnbull AR, Murphy R, Behrends V, Lund-Palau H, Simbo A, Mariveles M, Alton EWFW, Bush A, Shoemark A, Davies JC.

Am J Respir Crit Care Med. 2018 Jun 15;197(12):1635-1638. doi: 10.1164/rccm.201711-2365LE. No abstract available.


Current and future therapies for Pseudomonas aeruginosa infection in patients with cystic fibrosis.

Smith WD, Bardin E, Cameron L, Edmondson CL, Farrant KV, Martin I, Murphy RA, Soren O, Turnbull AR, Wierre-Gore N, Alton EW, Bundy JG, Bush A, Connett GJ, Faust SN, Filloux A, Freemont PS, Jones AL, Takats Z, Webb JS, Williams HD, Davies JC.

FEMS Microbiol Lett. 2017 Aug 1;364(14). doi: 10.1093/femsle/fnx121. Review.


Preparation for a first-in-man lentivirus trial in patients with cystic fibrosis.

Alton EW, Beekman JM, Boyd AC, Brand J, Carlon MS, Connolly MM, Chan M, Conlon S, Davidson HE, Davies JC, Davies LA, Dekkers JF, Doherty A, Gea-Sorli S, Gill DR, Griesenbach U, Hasegawa M, Higgins TE, Hironaka T, Hyndman L, McLachlan G, Inoue M, Hyde SC, Innes JA, Maher TM, Moran C, Meng C, Paul-Smith MC, Pringle IA, Pytel KM, Rodriguez-Martinez A, Schmidt AC, Stevenson BJ, Sumner-Jones SG, Toshner R, Tsugumine S, Wasowicz MW, Zhu J.

Thorax. 2017 Feb;72(2):137-147. doi: 10.1136/thoraxjnl-2016-208406. Epub 2016 Nov 16.


Genetic medicines for CF: Hype versus reality.

Alton EW, Boyd AC, Davies JC, Gill DR, Griesenbach U, Harrison PT, Henig N, Higgins T, Hyde SC, Innes JA, Korman MS.

Pediatr Pulmonol. 2016 Oct;51(S44):S5-S17. doi: 10.1002/ppul.23543. Review.


Cystic fibrosis gene therapy: a mutation-independent treatment.

Griesenbach U, Davies JC, Alton E.

Curr Opin Pulm Med. 2016 Nov;22(6):602-9. doi: 10.1097/MCP.0000000000000327. Review.


Pseudomonas aeruginosa infection in cystic fibrosis: pathophysiological mechanisms and therapeutic approaches.

Lund-Palau H, Turnbull AR, Bush A, Bardin E, Cameron L, Soren O, Wierre-Gore N, Alton EW, Bundy JG, Connett G, Faust SN, Filloux A, Freemont P, Jones A, Khoo V, Morales S, Murphy R, Pabary R, Simbo A, Schelenz S, Takats Z, Webb J, Williams HD, Davies JC.

Expert Rev Respir Med. 2016 Jun;10(6):685-97. doi: 10.1080/17476348.2016.1177460. Epub 2016 May 13. Review.


Does mass spectrometric breath analysis detect Pseudomonas aeruginosa in cystic fibrosis?

Pabary R, Huang J, Kumar S, Alton EW, Bush A, Hanna GB, Davies JC.

Eur Respir J. 2016 Mar;47(3):994-7. doi: 10.1183/13993003.00944-2015. Epub 2016 Feb 4. No abstract available.


A Phase I/IIa Safety and Efficacy Study of Nebulized Liposome-mediated Gene Therapy for Cystic Fibrosis Supports a Multidose Trial.

Alton EW, Boyd AC, Porteous DJ, Davies G, Davies JC, Griesenbach U, Higgins TE, Gill DR, Hyde SC, Innes JA; UK Cystic Fibrosis Gene Therapy Consortium *.

Am J Respir Crit Care Med. 2015 Dec 1;192(11):1389-92. doi: 10.1164/rccm.201506-1193LE. No abstract available.


Antipseudomonal Bacteriophage Reduces Infective Burden and Inflammatory Response in Murine Lung.

Pabary R, Singh C, Morales S, Bush A, Alshafi K, Bilton D, Alton EW, Smithyman A, Davies JC.

Antimicrob Agents Chemother. 2015 Nov 16;60(2):744-51. doi: 10.1128/AAC.01426-15. Print 2016 Feb.


Ex Vivo and In Vivo Lentivirus-Mediated Transduction of Airway Epithelial Progenitor Cells.

Leoni G, Wasowicz MY, Chan M, Meng C, Farley R, Brody SL, Inoue M, Hasegawa M, Alton EW, Griesenbach U.

Curr Gene Ther. 2015;15(6):581-90.


Multiple breath washouts in children can be shortened without compromising quality.

Ahmad F, Irving S, Alton E, Davies JC, Macleod K, Rosenthal M, Saunders C, Bush A, Saglani S, Fleming L.

Eur Respir J. 2015 Dec;46(6):1814-6. doi: 10.1183/13993003.00791-2015. Epub 2015 Oct 9. No abstract available.


Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial.

Alton EWFW, Armstrong DK, Ashby D, Bayfield KJ, Bilton D, Bloomfield EV, Boyd AC, Brand J, Buchan R, Calcedo R, Carvelli P, Chan M, Cheng SH, Collie DDS, Cunningham S, Davidson HE, Davies G, Davies JC, Davies LA, Dewar MH, Doherty A, Donovan J, Dwyer NS, Elgmati HI, Featherstone RF, Gavino J, Gea-Sorli S, Geddes DM, Gibson JSR, Gill DR, Greening AP, Griesenbach U, Hansell DM, Harman K, Higgins TE, Hodges SL, Hyde SC, Hyndman L, Innes JA, Jacob J, Jones N, Keogh BF, Limberis MP, Lloyd-Evans P, Maclean AW, Manvell MC, McCormick D, McGovern M, McLachlan G, Meng C, Montero MA, Milligan H, Moyce LJ, Murray GD, Nicholson AG, Osadolor T, Parra-Leiton J, Porteous DJ, Pringle IA, Punch EK, Pytel KM, Quittner AL, Rivellini G, Saunders CJ, Scheule RK, Sheard S, Simmonds NJ, Smith K, Smith SN, Soussi N, Soussi S, Spearing EJ, Stevenson BJ, Sumner-Jones SG, Turkkila M, Ureta RP, Waller MD, Wasowicz MY, Wilson JM, Wolstenholme-Hogg P; UK Cystic Fibrosis Gene Therapy Consortium.

Lancet Respir Med. 2015 Sep;3(9):684-691. doi: 10.1016/S2213-2600(15)00245-3. Epub 2015 Jul 3. Erratum in: Lancet Respir Med. 2015 Sep;3(9):e33.


Recent advances in understanding and managing cystic fibrosis transmembrane conductance regulator dysfunction.

Griesenbach U, Alton EW.

F1000Prime Rep. 2015 May 27;7:64. doi: 10.12703/P7-64. eCollection 2015. Review.


Cystic Fibrosis Gene Therapy in the UK and Elsewhere.

Griesenbach U, Pytel KM, Alton EW.

Hum Gene Ther. 2015 May;26(5):266-75. doi: 10.1089/hum.2015.027. Review.


Cyanide levels found in infected cystic fibrosis sputum inhibit airway ciliary function.

Nair C, Shoemark A, Chan M, Ollosson S, Dixon M, Hogg C, Alton EW, Davies JC, Williams HD.

Eur Respir J. 2014 Nov;44(5):1253-61. doi: 10.1183/09031936.00097014. Epub 2014 Sep 3.


Lung clearance index in primary ciliary dyskinesia and bronchiectasis.

Irving SJ, Davies JC, Alton EW, Bush A.

Am J Respir Crit Care Med. 2014 May 1;189(9):1147-8. doi: 10.1164/rccm.201402-0206LE. No abstract available.


Gene therapy in cystic fibrosis.

Armstrong DK, Cunningham S, Davies JC, Alton EW.

Arch Dis Child. 2014 May;99(5):465-8. doi: 10.1136/archdischild-2012-302158. Epub 2014 Jan 24. Review.


Toxicology study assessing efficacy and safety of repeated administration of lipid/DNA complexes to mouse lung.

Alton EW, Boyd AC, Cheng SH, Davies JC, Davies LA, Dayan A, Gill DR, Griesenbach U, Higgins T, Hyde SC, Innes JA, McLachlan G, Porteous D, Pringle I, Scheule RK, Sumner-Jones S.

Gene Ther. 2014 Jan;21(1):89-95. doi: 10.1038/gt.2013.61. Epub 2013 Nov 7.


The safety profile of a cationic lipid-mediated cystic fibrosis gene transfer agent following repeated monthly aerosol administration to sheep.

Alton EW, Baker A, Baker E, Boyd AC, Cheng SH, Coles RL, Collie DD, Davidson H, Davies JC, Gill DR, Gordon C, Griesenbach U, Higgins T, Hyde SC, Innes JA, McCormick D, McGovern M, McLachlan G, Porteous DJ, Pringle I, Scheule RK, Shaw DJ, Smith S, Sumner-Jones SG, Tennant P, Vrettou C.

Biomaterials. 2013 Dec;34(38):10267-77. doi: 10.1016/j.biomaterials.2013.09.023. Epub 2013 Oct 3.


Moving forward: cystic fibrosis gene therapy.

Griesenbach U, Alton EW.

Hum Mol Genet. 2013 Oct 15;22(R1):R52-8. doi: 10.1093/hmg/ddt372. Epub 2013 Aug 4. Review.


Lung clearance index and high-resolution computed tomography scores in primary ciliary dyskinesia.

Irving SJ, Ives A, Davies G, Donovan J, Edey AJ, Gill SS, Nair A, Saunders C, Wijesekera NT, Alton EW, Hansell D, Hogg C, Davies JC, Bush A.

Am J Respir Crit Care Med. 2013 Sep 1;188(5):545-9. doi: 10.1164/rccm.201304-0800OC.


Self-reactive CFTR T cells in humans: implications for gene therapy.

Calcedo R, Griesenbach U, Dorgan DJ, Soussi S, Boyd AC, Davies JC, Higgins TE, Hyde SC, Gill DR, Innes JA, Porteous DJ, Alton EW, Wilson JM, Limberis MP.

Hum Gene Ther Clin Dev. 2013 Sep;24(3):108-15. doi: 10.1089/humc.2012.249. Epub 2013 Jul 19.


A molecular comparison of microbial communities in bronchiectasis and cystic fibrosis.

Duff RM, Simmonds NJ, Davies JC, Wilson R, Alton EW, Pantelidis P, Cox MJ, Cookson WO, Bilton D, Moffatt MF.

Eur Respir J. 2013 Apr;41(4):991-3. doi: 10.1183/09031936.00052712. No abstract available.


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, Cunningham S, Davies JC, Gill DR, Griesenbach U, Higgins T, Hyde SC, Innes JA, Murray GD, Porteous DJ.

Thorax. 2013 Nov;68(11):1075-7. doi: 10.1136/thoraxjnl-2013-203309. Epub 2013 Mar 22.


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.

Thorax. 2013 Jun;68(6):532-9. doi: 10.1136/thoraxjnl-2012-202538. Epub 2013 Feb 9.


Expert opinion in biological therapy: update on developments in lung gene transfer.

Griesenbach U, Alton EW.

Expert Opin Biol Ther. 2013 Mar;13(3):345-60. doi: 10.1517/14712598.2013.735656. Epub 2013 Jan 5. Review.


Assessment of F/HN-pseudotyped lentivirus as a clinically relevant vector for lung gene therapy.

Griesenbach U, Inoue M, Meng C, Farley R, Chan M, Newman NK, Brum A, You J, Kerton A, Shoemark A, Boyd AC, Davies JC, Higgins TE, Gill DR, Hyde SC, Innes JA, Porteous DJ, Hasegawa M, Alton EW.

Am J Respir Crit Care Med. 2012 Nov 1;186(9):846-56. doi: 10.1164/rccm.201206-1056OC. Epub 2012 Sep 6.


Clinical expert panel on monitoring potential lung toxicity of inhaled oligonucleotides: consensus points and recommendations.

Alton EW, Boushey HA, Garn H, Green FH, Hodges M, Martin RJ, Murdoch RD, Renz H, Shrewsbury SB, Seguin R, Johnson G, Parry JD, Tepper J, Renzi P, Cavagnaro J, Ferrari N.

Nucleic Acid Ther. 2012 Aug;22(4):246-54. doi: 10.1089/nat.2012.0345. Epub 2012 Jul 18.


Assessment of the nuclear pore dilating agent trans-cyclohexane-1,2-diol in differentiated airway epithelium.

Griesenbach U, Wilson KM, Farley R, Meng C, Munkonge FM, Cheng SH, Scheule RK, Alton EW.

J Gene Med. 2012 Jul;14(7):491-500. doi: 10.1002/jgm.2643.


Oral contraceptives do not appear to affect cystic fibrosis disease severity.

Kernan NG, Alton EW, Cullinan P, Griesenbach U, Bilton D.

Eur Respir J. 2013 Jan;41(1):67-73. doi: 10.1183/09031936.00018712. Epub 2012 May 3.


Progress in gene and cell therapy for cystic fibrosis lung disease.

Griesenbach U, Alton EW.

Curr Pharm Des. 2012;18(5):642-62. Review.


Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis.

Regamey N, Tsartsali L, Hilliard TN, Fuchs O, Tan HL, Zhu J, Qiu YS, Alton EW, Jeffery PK, Bush A, Davies JC.

Thorax. 2012 Feb;67(2):164-70. doi: 10.1136/thoraxjnl-2011-200585. Epub 2011 Oct 18.


Differential global gene expression in cystic fibrosis nasal and bronchial epithelium.

Ogilvie V, Passmore M, Hyndman L, Jones L, Stevenson B, Wilson A, Davidson H, Kitchen RR, Gray RD, Shah P, Alton EW, Davies JC, Porteous DJ, Boyd AC.

Genomics. 2011 Nov;98(5):327-36. doi: 10.1016/j.ygeno.2011.06.008. Epub 2011 Jul 2.


Design of gene therapy trials in CF patients.

Davies JC, Alton EW.

Methods Mol Biol. 2011;741:55-68. doi: 10.1007/978-1-61779-117-8_5. Review.


Pre-clinical evaluation of three non-viral gene transfer agents for cystic fibrosis after aerosol delivery to the ovine lung.

McLachlan G, Davidson H, Holder E, Davies LA, Pringle IA, Sumner-Jones SG, Baker A, Tennant P, Gordon C, Vrettou C, Blundell R, Hyndman L, Stevenson B, Wilson A, Doherty A, Shaw DJ, Coles RL, Painter H, Cheng SH, Scheule RK, Davies JC, Innes JA, Hyde SC, Griesenbach U, Alton EW, Boyd AC, Porteous DJ, Gill DR, Collie DD.

Gene Ther. 2011 Oct;18(10):996-1005. doi: 10.1038/gt.2011.55. Epub 2011 Apr 21.


Current status and future directions of gene and cell therapy for cystic fibrosis.

Griesenbach U, Alton EW.

BioDrugs. 2011 Apr 1;25(2):77-88. doi: 10.2165/11586960-000000000-00000. Review.


Secreted Gaussia luciferase as a sensitive reporter gene for in vivo and ex vivo studies of airway gene transfer.

Griesenbach U, Vicente CC, Roberts MJ, Meng C, Soussi S, Xenariou S, Tennant P, Baker A, Baker E, Gordon C, Vrettou C, McCormick D, Coles R, Green AM, Lawton AE, Sumner-Jones SG, Cheng SH, Scheule RK, Hyde SC, Gill DR, Collie DD, McLachlan G, Alton EW.

Biomaterials. 2011 Apr;32(10):2614-24. doi: 10.1016/j.biomaterials.2010.12.001. Epub 2011 Jan 15.


Stem cell infusion into the vein of Marshall.

Lyne JC, Green AC, Alton EW, Clague JR.

Europace. 2011 Mar;13(3):438. doi: 10.1093/europace/euq373. Epub 2010 Dec 20.


Gene therapy for cystic fibrosis.

Davies JC, Alton EW.

Proc Am Thorac Soc. 2010 Nov;7(6):408-14. doi: 10.1513/pats.201004-029AW. Review.


Validation of recombinant Sendai virus in a non-natural host model.

Griesenbach U, McLachlan G, Owaki T, Somerton L, Shu T, Baker A, Tennant P, Gordon C, Vrettou C, Baker E, Collie DD, Hasegawa M, Alton EW.

Gene Ther. 2011 Feb;18(2):182-8. doi: 10.1038/gt.2010.131. Epub 2010 Oct 21.


Airway remodelling and its relationship to inflammation in cystic fibrosis.

Regamey N, Jeffery PK, Alton EW, Bush A, Davies JC.

Thorax. 2011 Jul;66(7):624-9. doi: 10.1136/thx.2009.134106. Epub 2010 Oct 1. Review.


Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator function.

Simmonds NJ, D'Souza L, Roughton M, Alton EW, Davies JC, Hodson ME.

Eur Respir J. 2011 May;37(5):1076-82. doi: 10.1183/09031936.00079010. Epub 2010 Sep 16.


Cystic fibrosis gene therapy: successes, failures and hopes for the future.

Griesenbach U, Alton EW.

Expert Rev Respir Med. 2009 Aug;3(4):363-71. doi: 10.1586/ers.09.25.


Quantification of periciliary fluid height in human airway biopsies is feasible, but not suitable as a biomarker.

Griesenbach U, Soussi S, Larsen MB, Casamayor I, Dewar A, Regamey N, Bush A, Shah PL, Davies JC, Alton EW.

Am J Respir Cell Mol Biol. 2011 Mar;44(3):309-15. doi: 10.1165/rcmb.2009-0265OC. Epub 2010 Apr 23.


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