E. Alton

发表

Enhanced lung gene expression after aerosol delivery of concentrated pDNA/PEI complexes.

Christina Vrettou, Peter Tennant, Alison Baker, 2008, Molecular therapy : the journal of the American Society of Gene Therapy.

Barriers to and new approaches for gene therapy and gene delivery in cystic fibrosis

D. Geddes, E. Alton, S. Ferrari, 2002, Advanced Drug Delivery Reviews.

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

M. Hodson, M. Roughton, J. Davies, 2010, European Respiratory Journal.

Cystic fibrosis and survival to 40 years: a study of CFTR function

M. Hodson, M. Roughton, J. Davies, 2010 .

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

J. Davies, E. Alton, C. Guest, 2019, European Respiratory Journal.

Transepithelial nasal potential difference in patients with, and at risk of acute respiratory distress syndrome

M. Matthay, D. McAuley, R. Verghis, 2021, Thorax.

Gene therapy for cystic fibrosis: steady progress, should do well.

D. Geddes, E. Alton, 1997, The European respiratory journal.

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

G. Hanna, A. Bush, J. Davies, 2016, European Respiratory Journal.

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

A. Gaggar, J. Blalock, A. Bush, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Outcome of clinical trials: Electricity, induced sputum, and breath

J. Davies, E. Alton, M. Waller, 2015 .

How Could We Do Better ?

E. Alton, 2009 .

Monitoring respiratory disease severity in cystic fibrosis.

J. Davies, E. Alton, 2009, Respiratory care.

Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence.

D. Geddes, M. Evans, W. Colledge, 2001, The European respiratory journal.

Differential binding of mannose-binding lectin to respiratory pathogens in cystic fibrosis

N. Klein, M. Turner, J. Davies, 2000, The Lancet.

Basic science: What will it deliver? Gene and stem cell therapy

E. Alton, U. Griesenbach, 2015 .

Gene therapy for cystic fibrosis

D. Geddes, E. Alton, StephenE. Smith, 1997, The Lancet.

In Vivo Measurement of Airway Potential Difference to Assess CFTR Function in Man

E. Alton, P. Middleton, 2006 .

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

P. Freemont, S. Faust, J. Bundy, 2016, Expert review of respiratory medicine.

Heart-lung transplantation for cystic fibrosis.

M. Yacoub, D. Geddes, M. Hodson, 1992, BMJ.

Identification and Functional Characterization of Cytoplasmic Determinants of Plasmid DNA Nuclear Import*

D. Geddes, M. Ward, M. Dunn, 2009, The Journal of Biological Chemistry.

Identification of protein cofactors necessary for sequence-specific plasmid DNA nuclear import.

Aaron M. Miller, D. Dean, E. Alton, 2009, Molecular therapy : the journal of the American Society of Gene Therapy.

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

R. Scheule, Seng H. Cheng, E. Alton, 2012, The journal of gene medicine.

TITLE Towards Gene Therapy for Cystic Fibrosis Using a Lentivirus Pseudotyped With Sendai Virus Envelopes

K. Mitomo, Y. Ueda, M. Hasegawa, 2015 .

Moving forward: cystic fibrosis gene therapy.

E. Alton, U. Griesenbach, 2013, Human molecular genetics.

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

D. Porteous, J. Davies, M. Hasegawa, 2012, American journal of respiratory and critical care medicine.

Detection of CFTR transgene mRNA expression in respiratory epithelium isolated from the murine nasal cavity

D. Porteous, R. Scheule, Seng H. Cheng, 2010, The journal of gene medicine.

Exploring the mechanisms of macrolides in cystic fibrosis.

D. Geddes, A. Bush, J. Davies, 2006, Respiratory medicine.

Gene Therapy for Lung Diseases

D. Geddes, J. Davies, E. Alton, 2006, Principles of Molecular Medicine.

Pulmonary Gene Therapy

D. Geddes, J. Davies, E. Alton, 2003 .

Antipseudomonal Bacteriophage Reduces Infective Burden and Inflammatory Response in Murine Lung

A. Bush, J. Davies, E. Alton, 2015, Antimicrobial Agents and Chemotherapy.

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

P. Shah, A. Bush, J. Davies, 2011, American journal of respiratory cell and molecular biology.

Gene therapy progress and prospects: cystic fibrosis

E. Alton, D. Geddes, U. Griesenbach, 2002, Gene Therapy.

In vivo transfer of bacterial marker genes results in differing levels of gene expression and tumor progression in immunocompetent and immunodeficient mice.

D. Geddes, O. Pardo, E. Alton, 1999, Human gene therapy.

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

L. Davies, S. Hyde, I. Pringle, 2018, Gene Therapy.

Evaluation of the porcine ameroid constrictor model of myocardial ischemia for therapeutic angiogenesis studies.

D. Geddes, Kaori Sato, P. Radke, 2006, Endothelium : journal of endothelial cell research.

Critical appraisal of the mouse model of myocardial infarction

D. Geddes, Kaori Sato, Jie Zhu, 2004, Experimental physiology.

Effects of intramyocardial pVEGF165 delivery on regional myocardial blood flow: evidence for a spatial ‘delivery–efficacy’ mismatch

P. Radke, E. Alton, S. Ferrari, 2004, Gene Therapy.

Efficient gene transfer to airway epithelium using recombinant Sendai virus

D. Geddes, Y. Yonemitsu, Y. Nagai, 2000, Nature Biotechnology.

Cardiac Repair Clinical Trials

E. Alton, Amanda C. Green, A. C. Green, 2008 .

Stem cell delivery modalities

E. Alton, J. Clague, A. Heinl-Green, 2006 .

Intravenously administered oligonucleotides can be delivered to conducting airway epithelium via the bronchial circulation

D. Rogers, E. Alton, L. Huang, 2006, Gene Therapy.

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

A. Bush, Z. Takáts, F. Bolt, 2018, Scientific Reports.

The use of carboxymethylcellulose gel to increase non-viral gene transfer in mouse airways.

R. Scheule, Seng H. Cheng, P. Sinn, 2010, Biomaterials.

Lung clearance index in CF: a sensitive marker of lung disease severity

J. Davies, E. Alton, S. Cunningham, 2007, Thorax.

Using magnetic forces to enhance non-viral gene transfer to airway epithelium in vivo

D. Geddes, R. Scheule, C. Plank, 2006, Gene Therapy.

Preparation for a fi rst-inman lentivirus trial in patients with cystic fi brosis

A. Doherty, T. Maher, A. Rodriguez-Martinez, 2016 .

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

A. Doherty, T. Maher, A. Rodriguez-Martinez, 2016, Thorax.

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

J. Davies, E. Alton, A. Shoemark, 2014, European Respiratory Journal.

Inefficient cationic lipid-mediated siRNA and antisense oligonucleotide transfer to airway epithelial cells in vivo

N. Caplen, R. Scheule, Seng H. Cheng, 2006, Respiratory research.

Bone marrow stem cells do not repopulate the healthy upper respiratory tract

M. Rosenthal, D. Geddes, A. Bush, 2002, Pediatric pulmonology.

Pseudomonas aeruginosa induces p38MAP kinase-dependent IL-6 and CXCL8 release from bronchial epithelial cells via a Syk kinase pathway

Kazuhiro Ito, J. Davies, E. Alton, 2021, PloS one.

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

M. J. Roberts, R. Scheule, Seng H. Cheng, 2011, Biomaterials.

Effect of inhaled furosemide on metabisulfite- and methacholine-induced bronchoconstriction and nasal potential difference in asthmatic subjects.

K. Chung, D. Geddes, P. Barnes, 1990, The American review of respiratory disease.

Effect of platelet activating factor on formation and composition of airway fluid in the guinea‐pig trachea.

P. Boschetto, P. Barnes, D. Rogers, 1990, The Journal of physiology.

Transfection efficiency and toxicity following delivery of naked plasmid DNA and cationic lipid-DNA complexes to ovine lung segments.

D. Porteous, Seng H. Cheng, S. Hyde, 2003, Molecular therapy : the journal of the American Society of Gene Therapy.

Anti-inflammatory gene therapy directed at the airway epithelium

D. Geddes, Leaf Huang, E. Alton, 2000, Gene Therapy.

CFTR gene transfer reduces the binding of Pseudomonas aeruginosa to cystic fibrosis respiratory epithelium.

N. Caplen, D. Geddes, T. Pitt, 1996, American journal of respiratory cell and molecular biology.

Distinct pattern of inflammation in bronchoalveolar lavage and bronchial mucosa of children with cystic fibrosis Online Repository

A. Bush, J. Davies, Jie Zhu, 2011 .

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

H. Boushey, Richard J Martin, H. Renz, 2012, Nucleic acid therapeutics.

Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis.

D. Geddes, E. Alton, E. Alton, 1987, Thorax.

Upper respiratory tract viral infection and mucociliary clearance.

D. Geddes, D. Tyrrell, E. Alton, 1987, European journal of respiratory diseases.

Measurement of halide efflux from cultured and primary airway epithelial cells using fluorescence indicators.

G. Mclachlan, E. Alton, A. Edelman, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

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

M. Rosenthal, A. Bush, J. Davies, 2018, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Asthma prophylaxis agents alter the function of an airway epithelial chloride channel.

D. Geddes, E. Alton, D. Gruenert, 1996, American journal of respiratory cell and molecular biology.

Impaired stimulus-evoked mucus secretion in cystic fibrosis bronchi.

D. Rogers, E. Alton, A. Dewar, 1993, Experimental lung research.

Chloride transport and the action of sodium cromoglycate and nedocromil sodium in asthma

E. Alton, A. Norris, 1996, Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology.

The effect of mucolytic agents on gene transfer across a CF sputum barrier in vitro

E. Alton, F. Sorgi, L. Huang, 1998, Gene Therapy.

No added benefit from nebulized amiloride in patients with cystic fibrosis.

D. Geddes, M. Hodson, E. Alton, 1993, The European respiratory journal.

The role of doxorubicin in non-viral gene transfer in the lung.

R. Scheule, Seng H. Cheng, E. Alton, 2009, Biomaterials.

Expression and maturation of Sendai virus vector-derived CFTR protein: functional and biochemical evidence using a GFP-CFTR fusion protein

M. Hasegawa, E. Alton, A. Iida, 2007, Gene Therapy.

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

E. Alton, U. Griesenbach, 2015, F1000prime reports.

Airway remodelling in children with cystic fibrosis

A. Nicholson, A. Bush, J. Davies, 2007, Thorax.

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

A. Bush, J. Davies, E. Alton, 2019, ERJ Open Research.

The pharmacokinetics of uniphyllin in nocturnal asthma.

I. D. Johnston, M. Hetzel, E. Alton, 1986, British journal of diseases of the chest.

Gene therapy for asthma: inspired research or unnecessary effort?

D. Geddes, E. Alton, U. Griesenbach, 1999, Gene Therapy.

Airway remodelling and its relationship to inflammation in cystic fibrosis

A. Bush, J. Davies, E. Alton, 2010, Thorax.

THORAXJNL134106 1..6

A. Bush, J. Davies, E. Alton, 2022 .

The efficacy of a 'master switch gene' HIF-1alpha in a porcine model of chronic myocardial ischaemia.

D. Geddes, P. Radke, Jie Zhu, 2005, European heart journal.

Effect of amiloride and saline on nasal mucociliary clearance and potential difference in cystic fibrosis and normal subjects.

D. Geddes, E. Alton, P. Middleton, 1993, Thorax.

UK cystic fibrosis gene therapy consortium tracking study: Exhaled breath condensate pH increases with treatment of an infective exacerbation

D. Porteous, D. Geddes, R. Gray, 2007 .

Progress and Prospects: Gene Therapy Clinical Trials (Part 1)

E. Alton, 2007, Gene Therapy.

Variability in Bacteriophage and Antibiotic Sensitivity in Serial Pseudomonas aeruginosa Isolates from Cystic Fibrosis Airway Cultures over 12 Months

J. Davies, E. Alton, S. Morales, 2021, Microorganisms.

Sendai virus-mediated CFTR gene transfer to the airway epithelium

A. M. Wright, D. Geddes, B. Wainwright, 2007, Gene Therapy.

Gene Therapy Progress and Prospects: Cystic fibrosis

D. Geddes, E. Alton, S. Ferrari, 2002, Gene Therapy.

Identification and validation of a novel pathogenic variant in GDF2 (BMP9) responsible for hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations

M. Caulfield, A. Devereau, C. Shovlin, 2021, American journal of medical genetics. Part A.

Immunological hurdles to lung gene therapy

D. Geddes, E. Alton, S. Ferrari, 2003, Clinical and experimental immunology.

CpG-free plasmids confer reduced inflammation and sustained pulmonary gene expression

David N Sheppard, Uta Griesenbach, David J Porteous, 2008, Nature Biotechnology.

Progress in Respiratory Gene Therapy

J. Davies, S. Hyde, D. Gill, 2022, Human gene therapy.

Cystic fibrosis gene therapy: a mutation-independent treatment

J. Davies, E. Alton, U. Griesenbach, 2016, Current opinion in pulmonary medicine.

Gene therapy for cystic fibrosis: recent progress and current aims

E. Alton, U. Griesenbach, Robyn V. Bell, 2016 .

Preparation for a ﬁ rst-in-man lentivirus trial in patients with cystic ﬁ brosis

A. Doherty, T. Maher, A. Rodriguez-Martinez, 2016 .

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

D. Porteous, J. Davies, S. Hyde, 2015, American journal of respiratory and critical care medicine.

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

D. Porteous, R. Scheule, J. Davies, 2013, Gene Therapy.

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

D. Porteous, D. Shaw, R. Scheule, 2013, Biomaterials.

A randomised, double-blind, placebo-controlled phase IIB clinical trial of repeated application of gene therapy in patients with cystic fibrosis

D. Porteous, G. Murray, Seng H. Cheng, 2013, Thorax.

Design of gene therapy trials in CF patients.

J. Davies, E. Alton, 2011, Methods in molecular biology.

An immunocytochemical assay to detect human CFTR expression following gene transfer.

A. Nairn, D. Porteous, R. Gray, 2009, Molecular and cellular probes.

A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction.

D. Porteous, B. Wainwright, J. Dorin, 1996, Gene therapy.

Correction of a chronic pulmonary disease through lentiviral vector-mediated protein expression

S. Hyde, D. Gill, E. Alton, 2022, Molecular therapy. Methods & clinical development.

Physico-chemical analysis of cationic liposome–DNA complexes (lipoplexes) with respect to in vitro and in vivo gene delivery efficiency

Andrew D. Miller, L. Stewart, E. Alton, 2001 .

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

T. Shu, M. Hasegawa, G. Mclachlan, 2011, Gene Therapy.

Low-frequency ultrasound increases non-viral gene transfer to the mouse lung.

D. Geddes, R. Scheule, Seng H. Cheng, 2010, Acta biochimica et biophysica Sinica.

Multiple breath washouts in children can be shortened without compromising quality

M. Rosenthal, A. Bush, J. Davies, 2015, European Respiratory Journal.

The severe G480C cystic fibrosis mutation, when replicated in the mouse, demonstrates mistrafficking, normal survival and organ-specific bioelectrics.

Martin S. Taylor, D. Porteous, R. Willemsen, 2002, Human molecular genetics.

Erythromycin inhibits Cl secretion.

D. Geddes, E. Alton, 1992, The European respiratory journal.

Gene therapy for cystic fibrosis: a clinical perspective.

D. Geddes, E. Alton, 1995, Gene therapy.

Biomarkers for cystic fibrosis: are we progressing?

D. Geddes, J. Davies, E. Alton, 2007, American journal of respiratory and critical care medicine.

Nondisruptive, sequence-specific coupling of fluorochromes to plasmid DNA.

D. Dean, E. Alton, F. Munkonge, 2006, Analytical biochemistry.

Cytoplasmic deposition of NFκB decoy oligonucleotides is insufficient to inhibit bleomycin-induced pulmonary inflammation

E. Alton, R. Cassady, D. Geddes, 2002, Gene Therapy.

Characterization and comparison of ion transport across sheep and human airway epithelium.

D. Geddes, E. Alton, A. Graham, 1994, Epithelial cell biology.

Increased airway smooth muscle mass in children with asthma, cystic fibrosis and bronchiectasis

M. Ochs, A. Bush, J. Davies, 2008 .

Effects of 5-hydroxytryptamine and 5-hydroxytryptamine receptor agonists on ion transport across mammalian airway epithelia.

D. Geddes, E. Alton, A. Graham, 1992, Clinical science.

Prospects for gene therapy in cystic fibrosis

D. Geddes, A. Bush, E. Alton, 1999, Molecular medicine today.

Oral contraceptives do not appear to affect cystic fibrosis disease severity

P. Cullinan, E. Alton, D. Bilton, 2012, European Respiratory Journal.

Validation of nasal potential difference measurements in gut-corrected CF knockout mice.

Charanjit Singh, E. Alton, R. Farley, 2008, American journal of respiratory cell and molecular biology.

CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus vector

A. Mehta, P. Hegyi, Z. Rakonczay, 2008 .

Update on Preclinical and Clinical Gene Therapy for the Treatment of Cystic Fibrosis

E. Alton, U. Griesenbach, 2015 .

The Pathogenesis of Cystic Fibrosis and Progress Towards Gene Therapy

E. Alton, M. Stern, 1999, Journal of the Royal College of Physicians of London.

Prospects for gene therapy in cystic fibrosis

D. Geddes, A. Bush, E. Alton, 1999, Archives of disease in childhood.

Gene therapy for cystic fibrosis: which postman, which box?

E. Alton, P. Middleton, 1998, Thorax.

Gene therapy for cystic fibrosis.

D. Geddes, E. Alton, 1995, The Netherlands journal of medicine.

What the papers say: Cystic fibrosis: Prospects for therapy

D. Porteous, E. Alton, 1993 .

Optimizing aerosol gene delivery and expression in the ovine lung.

D. Porteous, R. Scheule, Seng H. Cheng, 2007, Molecular therapy : the journal of the American Society of Gene Therapy.

Nasal abnormalities in cystic fibrosis mice independent of infection and inflammation.

A. Bush, B. Wainwright, J. Davies, 2008, American journal of respiratory cell and molecular biology.

Cystic fibrosis transmembrane conductance regulator protein: what is its role in cystic fibrosis?

E. Alton, P. Richardson, 1993, The European respiratory journal.

Advances in cystic fibrosis gene therapy

D. Geddes, E. Alton, U. Griesenbach, 2004, Current opinion in pulmonary medicine.

Milking gene therapy

D. Geddes, E. Alton, U. Griesenbach, 1998, Nature Medicine.

Quality, size, and composition of pediatric endobronchial biopsies in cystic fibrosis.

M. Rosenthal, A. Bush, J. Davies, 2007, Chest.

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

D. Porteous, James M. Wilson, J. Davies, 2013, Human gene therapy. Clinical development.

Characterisation of LMD virus-like nanoparticles self-assembled from cationic liposomes, adenovirus core peptide μ (mu) and plasmid DNA

F. Booy, N. Lemoine, A. Miller, 2002, Gene Therapy.

Nasal epithelial ion transport and genetic analysis of infertile men with congenital bilateral absence of the vas deferens.

D. Geddes, M. Hodson, E. Alton, 1993, Human molecular genetics.

Effects of purified Pseudomonas rhamnolipids on bioelectric properties of sheep tracheal epithelium.

D. Geddes, E. Alton, R. Wilson, 1993, Experimental lung research.

Towards gene therapy for cystic fibrosis: a clinical progress report

D. Gill, E. Alton, J. Innes, 1998, Gene Therapy.

A mixed message for cystic fibrosis gene therapy

D. Geddes, E. Alton, 1994, Nature Genetics.

The in vivo effects of milrinone on the airways of cystic fibrosis mice and human subjects.

D. Geddes, B. Wainwright, E. Alton, 1999, American journal of respiratory cell and molecular biology.

Quantitative fluorescence measurements of chloride secretion in native airway epithelium from CF and non-CF subjects.

E. Alton, F. Sorgi, L. Huang, 1995, Gene therapy.

Characterization of a Ca(2+)‐dependent anion channel from sheep tracheal epithelium incorporated into planar bilayers.

D. Geddes, E. Alton, A. Williams, 1991, Journal of Physiology.

New treatments for cystic fibrosis.

D. Geddes, E. Alton, N. Caplen, 1992, British medical bulletin.

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

Steve Cunningham, Joseph Jacob, Sarah Sheard, 2015 .

Cystic Fibrosis Gene Therapy – Not Low-hanging Fruit

E. Alton, U. Griesenbach, 2016 .

Potential difference measurements in the lower airway of children with and without cystic fibrosis.

J. Pepper, D. Geddes, L. Collins, 2005, American journal of respiratory and critical care medicine.

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

A. Doherty, T. Maher, A. Rodriguez-Martinez, 2016, Thorax.

Poly (D, L-lactide-co-glycolide)/DNA microspheres to facilitate prolonged transgene expression in airway epithelium in vitro, ex vivo and in vivo

D. Geddes, E. Alton, M. Stern, 2003, Gene Therapy.

Gene therapy for cystic fibrosis: an example for lung gene therapy

E. Alton, D. Geddes, U. Griesenbach, 2004, Gene therapy.

In vivo imaging of gene transfer to the respiratory tract.

D. Geddes, R. Scheule, Seng H. Cheng, 2008, Biomaterials.

Effect of tolerance induction to immunodominant T-cell epitopes of Sendai virus on gene expression following repeat administration to lung

D. Geddes, D. Altmann, R. Boyton, 2006, Gene Therapy.

Tracheal potential difference in the reserpine and isoproterenol rat models of cystic fibrosis.

D. Geddes, D. Rogers, E. Alton, 1990, Experimental lung research.

ABSENCE OF ELECTROCHEMICAL DEFECT OF CYSTIC FIBROSIS IN TRANSPLANTED LUNG

D. Geddes, M. Hodson, E. Alton, 1987, The Lancet.

Cystic fibrosis: towards the basic defect.

D. Geddes, E. Alton, 1988, The Quarterly journal of medicine.

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

P. Freemont, S. Faust, J. Bundy, 2017, FEMS microbiology letters.

Genetic medicines for CF: Hype versus reality

J. Davies, S. Hyde, D. Gill, 2016, Pediatric pulmonology.

Localization and up‐regulation of Mucin (MUC2) gene expression in human nasal biopsies of patients with cystic fibrosis

Dechun Li, S. Durham, N. Caplen, 1997, The Journal of pathology.

Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type Cftr gene expression

D. Porteous, J. Dorin, E. Alton, 1994, Mammalian Genome.

Cystic fibrosis in the mouse by targeted insertional mutagenesis

Wendy L. Kimber, David J. Porteous, Paul Dickinson, 1992, Nature.

Cystic Fibrosis Gene Therapy in the UK and Elsewhere

E. Alton, U. Griesenbach, K. Pytel, 2015, Human gene therapy.

Gene therapy in cystic fibrosis

J. Davies, E. Alton, S. Cunningham, 2014, Archives of Disease in Childhood.

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

E. Alton, U. Griesenbach, 2013, Expert opinion on biological therapy.

Viral vectors for cystic fibrosis gene therapy: What does the future hold?

M. Hasegawa, E. Alton, U. Griesenbach, 2010 .

Gene transfer to the lung: lessons learned from more than 2 decades of CF gene therapy.

E. Alton, U. Griesenbach, 2009, Advanced drug delivery reviews.

Use of ultrasound to enhance nonviral lung gene transfer in vivo

D. Geddes, R. Scheule, E. Alton, 2007, Gene Therapy.

The extra- and intracellular barriers to lipid and adenovirus-mediated pulmonary gene transfer in native sheep airway epithelium

D. Geddes, C. Kitson, N. Severs, 1999, Gene Therapy.

In vitro liposome-mediated DNA transfection of epithelial cell lines using the cationic liposome DC-Chol/DOPE.

N. Caplen, D. Geddes, E. Kinrade, 1995, Gene therapy.

Airway gene therapy.

J. Davies, E. Alton, 2005, Advances in genetics.

Therapeutic Approaches to the Lung Problems in Cystic Fibrosis

E. Alton, M. Stern, 1997 .

Beta-defensin genomic copy number is not a modifier locus for cystic fibrosis

J. Davies, E. Alton, E. Hollox, 2005, Journal of Negative Results in BioMedicine.

Chloride transport and the actions of nedocromil sodium and cromolyn sodium in asthma.

E. Alton, A. Norris, 1996, The Journal of allergy and clinical immunology.

Lack of inhibition by dideoxy-forskolin and verapamil of DIDS-sensitive volume-activated Cl- secretion in human squamous lung carcinoma epithelial cells.

D. Geddes, E. Alton, F. Munkonge, 1994, Biochimica et biophysica acta.

Toward gene therapy for cystic fibrosis using a lentivirus pseudotyped with Sendai virus envelopes.

Raymond Farley, Uta Griesenbach, Makoto Inoue, 2010, Molecular therapy : the journal of the American Society of Gene Therapy.

Biomarkers for cystic fibrosis lung disease: application of SELDI-TOF mass spectrometry to BAL fluid.

D. Porteous, R. Gray, A. Bush, 2008, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Pretreatment with cationic lipid-mediated transfer of the Na+K+-ATPase pump in a mouse model in vivo augments resolution of high permeability pulmonary oedema

E. Alton, S. Cheng, Y. Berthiaume, 2000, Gene Therapy.

Non-Viral Gene Therapy for Pulmonary Disease

E. Alton, M. Stern, 2001 .

Bactofection of lung epithelial cells in vitro and in vivo using a genetically modified Escherichia coli

D. Geddes, R. Scheule, E. Alton, 2008, Gene Therapy.

Prospects for respiratory gene therapy.

D. Geddes, E. Alton, 1997, British journal of hospital medicine.

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

D. Porteous, R. Kitchen, P. Shah, 2011, Genomics.

Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation

D. Hansell, D. Porteous, D. Ashby, 2013, Thorax.

Nasal application of the cationic liposome DC-Chol:DOPE does not alter ion transport, lung function or bacterial growth.

E. Alton, L. Huang, X. Gao, 1994, The European respiratory journal.

Adenovirus-mediated in utero expression of CFTR does not improve survival of CFTR knockout mice.

L. Davies, S. Hyde, G. Nunez-Alonso, 2008, Molecular therapy : the journal of the American Society of Gene Therapy.

Emerging significance of plasmid DNA nuclear import in gene therapy.

D. Dean, E. Alton, U. Griesenbach, 2003, Advanced drug delivery reviews.

Gene complementation of airway epithelium in the cystic fibrosis mouse is necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities.

D. Hume, R. Schreiber, K. Kunzelmann, 2002, Human molecular genetics.

Gene therapy for cystic fibrosis

C. Kitson, E. Alton, 2000, Expert opinion on investigational drugs.

Cystic fibrosis

A. Bush, J. Davies, E. Alton, 2007, BMJ : British Medical Journal.

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

E. Alton, U. Griesenbach, 2009, Expert review of respiratory medicine.

Cystic fibrosis: Ferreting with fibroblasts for cystic fibrosis

E. Alton, U. Griesenbach, 2009, Gene Therapy.

Biomarkers for cystic fibrosis lung disease

D. Porteous, R. Gray, A. Bush, 2016 .

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

A. Bush, J. Davies, E. Alton, 2018, American journal of respiratory and critical care medicine.

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

A. Bush, J. Davies, E. Alton, 2011, Thorax.

A molecular comparison of microbial communities in bronchiectasis and cystic fibrosis

M. Moffatt, J. Davies, E. Alton, 2013, European Respiratory Journal.

Increased airway smooth muscle mass in children with asthma, cystic fibrosis, and non-cystic fibrosis bronchiectasis.

M. Ochs, A. Bush, J. Davies, 2008, American journal of respiratory and critical care medicine.

Laboratory and clinical studies in support of cystic fibrosis gene therapy using pCMV-CFTR-DOTAP.

D. Porteous, G. Mclachlan, E. Alton, 1996, Gene therapy.

Use of Liposomes in the Treatment of Cystic Fibrosis

E. Alton, M. Stern, 2002 .

Effects of altering dosing on cationic liposome-mediated gene transfer to the respiratory epithelium

D. Geddes, E. Alton, S. Cheng, 1999, Gene Therapy.

Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: comparison with humans.

D. Porteous, D. Geddes, E. Alton, 1995, The American journal of physiology.

The CF gene: 10 years on

D. Geddes, E. Alton, 1999, Thorax.

Modification of gating of an airway epithelial chloride channel by 5-nitro-2-(3-phenylpropylamino)benzoic acid (NPPB)

A. J. Williams, E. Alton, 1992, The Journal of Membrane Biology.

Gene therapy for respiratory diseases; progress and a changing context.

J. Davies, S. Hyde, D. Gill, 2020, Human gene therapy.

Second‐messenger regulation of sodium transport in mammalian airway epithelia.

E. Alton, D. M. Geddes, A. Graham, 1992, The Journal of physiology.

Magnetic nanoparticles as gene delivery agents: enhanced transfection in the presence of oscillating magnet arrays

A. Keramane, C. Batich, J. Dobson, 2008, Nanotechnology.

Non–invasive liposome–mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice

A. Miller, S. Hart, E. Alton, 1993 .

Liposome-mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis

S. Durham, N. Caplen, D. Porteous, 1995, Nature Medicine.

Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium.

D. Geddes, E. Alton, E. Alton, 1994, The European respiratory journal.

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

E. Alton, U. Griesenbach, 2012, Current pharmaceutical design.

Recent progress in gene therapy for cystic fibrosis.

E. Alton, U. Griesenbach, 2001, Current opinion in molecular therapeutics.

Gene therapy for cystic fibrosis

D. Geddes, J. Davies, E. Alton, 2001, The journal of gene medicine.

Sendai virus for gene therapy and vaccination.

M. Hasegawa, E. Alton, U. Griesenbach, 2005, Current opinion in molecular therapeutics.

A defective nontransmissible recombinant Sendai virus mediates efficient gene transfer to airway epithelium in vivo

D. Geddes, T. Shu, M. Hasegawa, 2004, Gene Therapy.

Cationic lipid-mediated gene transfer to the growing murine and human airway

A. Bush, E. Alton, S. Cheng, 2000, Gene Therapy.

Assessment of CFTR function after gene transfer in vitro and in vivo.

S. Hyde, S. Sumner-Jones, D. Gill, 2008, Methods in molecular biology.

Cystic fibrosis--from gene to therapy.

D. Geddes, E. Alton, 1991, Transactions of the Medical Society of London.

The effects of jet nebulisation on cationic liposome-mediated gene transfer in vitro

E. Alton, F. Sorgi, D. Gruenert, 1998, Gene Therapy.

Nasal potential difference: a clinical diagnostic test for cystic fibrosis.

D. Geddes, M. Hodson, E. Alton, 1990, The European respiratory journal.

Trimethoprim and tetracycline inhibit airway epithelial sodium absorption.

D. Geddes, E. Alton, P. Middleton, 1996, American Journal of Respiratory and Critical Care Medicine.

The effect of varying tonicity on nasal epithelial ion transport in cystic fibrosis.

D. Geddes, E. Alton, M. Davies, 2005, American journal of respiratory and critical care medicine.

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

D. Hansell, A. Bush, J. Davies, 2013, American journal of respiratory and critical care medicine.

The introduction of two silent mutations into a CFTR cDNA construct allows improved detection of exogenous mRNA in gene transfer experiments.

S. Hart, E. Alton, C. Coutelle, 1995, Human Molecular Genetics.

Current Status and Future Directions of Gene and Cell Therapy for Cystic Fibrosis

E. Alton, U. Griesenbach, 2011, BioDrugs.

Cystic fibrosis in the 21st century

A. Bush, J. Davies, E. Alton, 2005 .

The nasal epithelium as a factory for systemic protein delivery.

D. Geddes, Y. Nagai, E. Schmitt, 2002, Molecular therapy : the journal of the American Society of Gene Therapy.

Effect of heart-lung transplantation on airway potential difference in patients with and without cystic fibrosis.

M. Yacoub, D. Geddes, E. Alton, 1991, The European respiratory journal.

Vascular oligonucleotide transfer facilitated by a polymer-coated stent.

D. Geddes, P. Radke, E. Alton, 2005, Human gene therapy.

T4 Safety and expression of a single dose of lipid-mediated CFTR gene therapy to the upper and lower airways of patients with Cystic Fibrosis

R. Scheule, S. Hyde, D. Gill, 2011, Thorax.

S48 Mucoidy and the microbiome: community composition in relation to the presence of culturable, mucoid Pseudomonas aeruginosa

A. Jones, J. Davies, E. Alton, 2011, Thorax.

Ion transport characteristics of the murine trachea and caecum.

D. Geddes, E. Alton, S. N. Smith, 1992, Clinical science.

Modifier genes in cystic fibrosis

J. Davies, E. Alton, U. Griesenbach, 2004, Pediatric pulmonology.

Pulmonary disease severity in men with ΔF508 cystic fibrosis and residual chloride secretion

D. Geddes, M. Hodson, E. Alton, 1999, The Lancet.

In vitro bioelectric properties of bronchial epithelium from transplanted lungs in recipients with cystic fibrosis.

M. Yacoub, M. Hodson, E. Alton, 1993, Thorax.

Bioelectric properties of cystic fibrosis airways obtained at heart-lung transplantation.

M. Yacoub, D. Geddes, D. Rogers, 1992, Thorax.

Update on gene therapy for cystic fibrosis.

D. Geddes, E. Alton, U. Griesenbach, 2003, Current opinion in molecular therapeutics.

Heat shock proteins in cancer therapy.

D. Geddes, O. Pardo, E. Alton, 2000, Advances in experimental medicine and biology.

Lytic Bacteriophage Is a Promising Adjunct to Common Antibiotics across Cystic Fibrosis Clinical Strains and Culture Models of Pseudomonas aeruginosa Infection

J. Davies, E. Alton, S. Morales, 2023, Antibiotics.

A randomised, double-blind, placebo-controlled trial of repeated nebulisation of non-viral cystic fibrosis transmembrane conductance regulator ( CFTR) gene therapy in patients with cystic fibrosis

A. Nicholson, D. Hansell, A. Maclean, 2016 .

Lack of effect of lung denervation on the measurement of potential difference after single-lung transplantation.

M. Yacoub, D. Geddes, E. Alton, 1989, New England Journal of Medicine.

Limitations of the murine nose in the development of nonviral airway gene transfer.

D. Porteous, Charanjit Singh, R. Scheule, 2010, American journal of respiratory cell and molecular biology.

Gene therapy for respiratory diseases: potential applications and difficulties.

D. Geddes, E. Alton, 1995, Thorax.

Nitrogen offset in N2 multiple washout method

A. Bush, J. Davies, E. Alton, 2020, ERJ Open Research.

Molecular Therapies for Cystic Fibrosis

J. Davies, E. Alton, G. Davies, 2019, Kendig's Disorders of the Respiratory Tract in Children.

A mild variant of cystic fibrosis.

E. Alton, E. W. Alton, 1996, Thorax.

Age-specific effect of the cystic fibrosis modifier gene, MBL-2

D. Geddes, N. Klein, M. Turner, 2002 .

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

L. Davies, S. Hyde, I. Pringle, 2018, Gene Therapy.

534. Preparation for a First-in-Man Lentivirus Trial in Cystic Fibrosis Patients

J. Davies, M. Hasegawa, L. Davies, 2016 .

Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype‐phenotype correlations.

D. Porteous, B. Wainwright, J. Dorin, 1996, The EMBO journal.

Prospects for gene therapy in lung disease.

D. Geddes, J. Davies, E. Alton, 2001, Current opinion in pharmacology.

Taking stock of gene therapy for cystic fibrosis

D. Geddes, E. Alton, M. Stern, 2000, Respiratory research.

Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial

S. Durham, D. Geddes, Seng H. Cheng, 1999, The Lancet.

Gene Therapy: The Case for Cystic Fibrosis

D. Geddes, E. Alton, 1997, Journal of the Royal Society of Medicine.

Gene therapy for cystic fibrosis

E. Alton, 1995, Journal of Inherited Metabolic Disease.

Towards Gene Therapy For Cystic Fibrosis *

E. Alton, 1995, The Journal of pharmacy and pharmacology.

Use of nonviral vectors for cystic fibrosis gene therapy.

E. Alton, Eric W F W Alton, 2004, Proceedings of the American Thoracic Society.

Cystic Fibrosis Gene Therapy

D. Geddes, E. Alton, U. Griesenbach, 1999 .

GENE DELIVERY AND THERAPY: THE CASE FOR CYSTIC FIBROSIS

E. Alton, 1996 .

Corrigendum: Non–invasive liposome–mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice

S. Hart, E. Alton, P. Jeffery, 1993, Nature Genetics.

Effect of IBMX and alkaline phosphatase inhibitors on Cl- secretion in G551D cystic fibrosis mutant mice.

D. Porteous, D. Geddes, B. Wainwright, 1998, American journal of physiology. Cell physiology.

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

A. Nicholson, D. Hansell, A. Maclean, 2015, The Lancet. Respiratory medicine.

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

D. Porteous, D. Shaw, R. Scheule, 2011, Gene Therapy.

Unsupervised home spirometry is not equivalent to supervised clinic spirometry in children and young people with cystic fibrosis: Results from the CLIMB‐CF study

M. Rosenthal, J. Wallenburg, E. Alton, 2023, Pediatric pulmonology.

Mannose-binding lectin is present in the infected airway: a possible pulmonary defence mechanism

D. Geddes, N. Klein, M. Turner, 2008, Thorax.

Gene therapy for cystic fibrosis.

J. Davies, E. Alton, 2010, Proceedings of the American Thoracic Society.

Airway surface liquid calcium modulates chloride permeability in the cystic fibrosis airway.

D. Geddes, J. Wheatley, E. Alton, 2003, American journal of respiratory and critical care medicine.

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

P. Freemont, S. Faust, J. Bundy, 2016, Expert review of respiratory medicine.

Airway remodelling and its relationship to inﬂammation in cystic ﬁbrosis

E. Alton, N. Regamey, Peter K Jeffery, 2022 .

Whole Gene Sequencing of CFTR Reveals a High Prevalence of the Intronic Variant c.3874-4522A>G in Cystic Fibrosis.

J. Davies, E. Alton, S. John, 2020, American journal of respiratory and critical care medicine.

Gene therapy for respiratory diseases: potential applications and difficulties.

D. Geddes, E. Alton, 1995, Thorax.

Advanced cryptogenic fibrosing alveolitis: preliminary report on treatment with cyclosporin A.

E. Alton, M. Turner-Warwick, M. Johnson, 1989, Respiratory medicine.

Detection of CFTR transgene mRNA expression in respiratory epithelium isolated from the murine nasal cavity

D. Porteous, R. Scheule, Seng H. Cheng, 2010, The journal of gene medicine.

Measurement of Halide Efflux from Cultured and Primary Airway Epithelial Cells Using Fluorescence Indicators

G. Mclachlan, E. Alton, A. Edelman, 2022 .

P u l m o n a ry disease severity in men with DF508 cystic fibrosis and residual chloride secre t i o n

D. Geddes, E. Alton, A. Jaffe, 1999 .

CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus vector.

A. Mehta, P. Hegyi, Z. Rakonczay, 2007, Journal of cellular physiology.

Endobronchial biopsy in childhood.

M. Rosenthal, A. Bush, J. Davies, 2008, Chest.

Safety and adverse events

A. Nicholson, D. Hansell, A. Maclean, 2016 .

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

M. Rosenthal, A. Bush, J. Davies, 2018, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

HVJ (Sendai virus) liposome-mediated gene transfer: current status and future perspectives (review).

K. Sugimachi, Y. Yonemitsu, Y. Kaneda, 1998, International journal of oncology.