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

Autor: EW Alton, A Khagani, RF Taylor, R Logan-Sinclair, M Yacoub, DM Geddes

Publikováno v: European Respiratory Journal. 4:5-9

Measurement of the potential difference (PD) across the airways provides an indication of the viability and integrity of the lining epithelium. PD was recorded from the lower airways in "diseased controls" and in patients following heart-lung transpl

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::3f60b8cdfce588853b3bd90d59671490
https://doi.org/10.1183/09031936.93.04010005

No added benefit from nebulized amiloride in patients with cystic fibrosis

Autor: A Graham, A Hasani, EW Alton, GP Martin, C Marriott, ME Hodson, SW Clarke, DM Geddes

Publikováno v: The European respiratory journal. 6(9)

In cystic fibrosis (CF) airway epithelial sodium absorption is increased 2-3 fold. Since sodium absorption is inhibited by the sodium channel blocker amiloride, our aim was to assess its therapeutic benefit in cystic fibrosis. A randomized, double-bl

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b2f76933b8e545bdbfabfefd7f7ecac9
https://pubmed.ncbi.nlm.nih.gov/8287938

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

Autor: McLachlan G, Ling Pei Ho, Davidson-Smith H, Samways J, Davidson H, Bj, Stevenson, Ad, Carothers, Ew, Alton, Pg, Middleton, Sn, Smith, Kallmeyer G, Michaelis U, Dj, Porteous

Publikováno v: Europe PubMed Central

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=dedup_wf_001::4ddba013d17b89f6ab418eebfc528673
http://europepmc.org/abstract/med/8986438

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

Autor: Balachandar S; Division of Pulmonary, Critical Care, Sleep and Occupational Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA., Graves TJ; Division of Pulmonary, Critical Care, Sleep and Occupational Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA., Shimonty A; Division of Pulmonary, Critical Care, Sleep and Occupational Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA., Kerr K; School of Medicine, Dentistry and Biomedical Sciences, Queen's University Belfast, Belfast, UK., Kilner J; School of Medicine, Dentistry and Biomedical Sciences, Queen's University Belfast, Belfast, UK., Xiao S; National Heart and Lung Institute, Imperial College London, London, UK.; Genomics England Respiratory Clinical Interpretation Partnership (GeCIP), London, UK., Slade R; National Heart and Lung Institute, Imperial College London, London, UK.; Genomics England Respiratory Clinical Interpretation Partnership (GeCIP), London, UK., Sroya M; Department of Surgery and Cancer, Imperial College London, London, UK., Alikian M; Genomics England Respiratory Clinical Interpretation Partnership (GeCIP), London, UK.; West London Genomic Medicine Centre, Imperial College Healthcare NHS Trust, London, UK., Curetean E; West London Genomic Medicine Centre, Imperial College Healthcare NHS Trust, London, UK., Thomas E; West London Genomic Medicine Centre, Imperial College Healthcare NHS Trust, London, UK.; Genomics England, London, UK., McConnell VPM; Regional Genetics Service, Belfast Health and Social Care Trust, Belfast, UK., McKee S; Regional Genetics Service, Belfast Health and Social Care Trust, Belfast, UK., Boardman-Pretty F; Genomics England, London, UK., Devereau A; Genomics England, London, UK., Fowler TA; Genomics England, London, UK.; William Harvey Research Institute, Queen Mary University of London, London, UK., Caulfield MJ; Genomics England, London, UK.; William Harvey Research Institute, Queen Mary University of London, London, UK., Alton EW; National Heart and Lung Institute, Imperial College London, London, UK.; Genomics England Respiratory Clinical Interpretation Partnership (GeCIP), London, UK., Ferguson T; West London Genomic Medicine Centre, Imperial College Healthcare NHS Trust, London, UK., Redhead J; West London Genomic Medicine Centre, Imperial College Healthcare NHS Trust, London, UK., McKnight AJ; School of Medicine, Dentistry and Biomedical Sciences, Queen's University Belfast, Belfast, UK.; Genomics England Respiratory Clinical Interpretation Partnership (GeCIP), London, UK., Thomas GA; Department of Surgery and Cancer, Imperial College London, London, UK., Aldred MA; Division of Pulmonary, Critical Care, Sleep and Occupational Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.; Genomics England Respiratory Clinical Interpretation Partnership (GeCIP), London, UK., Shovlin CL; National Heart and Lung Institute, Imperial College London, London, UK.; Genomics England Respiratory Clinical Interpretation Partnership (GeCIP), London, UK.; West London Genomic Medicine Centre, Imperial College Healthcare NHS Trust, London, UK.

Publikováno v: American journal of medical genetics. Part A [Am J Med Genet A] 2022 Mar; Vol. 188 (3), pp. 959-964. Date of Electronic Publication: 2021 Dec 13.

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

Autor: Alton EW; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Beekman JM; Department of Pediatric Pulmonology, Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Centre, Utrecht, The Netherlands., Boyd AC; Centre for Genomic and Experimental Medicine, IGMM, University of Edinburgh, Edinburgh, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Brand J; Centre for Genomic and Experimental Medicine, IGMM, University of Edinburgh, Edinburgh, UK.; Lung Pathology Unit, Department of Airway Disease Infection, NHLI, Imperial College London, London, UK., Carlon MS; Laboratory for Molecular Virology and Gene Therapy, Department of Pharmaceutical and Pharmacological Sciences, KU Leuven, Brussels, Belgium., Connolly MM; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Gene Medicine Research Group, NDCLS, John Radcliffe Hospital, Oxford, UK., Chan M; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Conlon S; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Davidson HE; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Centre for Genomic and Experimental Medicine, IGMM, University of Edinburgh, Edinburgh, UK., Davies JC; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Davies LA; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Gene Medicine Research Group, NDCLS, John Radcliffe Hospital, Oxford, UK., Dekkers JF; Department of Pediatric Pulmonology, Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Centre, Utrecht, The Netherlands., Doherty A; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Centre for Genomic and Experimental Medicine, IGMM, University of Edinburgh, Edinburgh, UK., Gea-Sorli S; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Gill DR; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Gene Medicine Research Group, NDCLS, John Radcliffe Hospital, Oxford, UK., Griesenbach U; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Hasegawa M; ID Pharme Co. Ltd. (DNAVEC Center), Tsukuba, Japan., Higgins TE; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Hironaka T; ID Pharme Co. Ltd. (DNAVEC Center), Tsukuba, Japan., Hyndman L; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Centre for Genomic and Experimental Medicine, IGMM, University of Edinburgh, Edinburgh, UK., McLachlan G; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Roslin Institute & R(D)SVS, University of Edinburgh, Midlothian, UK., Inoue M; ID Pharme Co. Ltd. (DNAVEC Center), Tsukuba, Japan., Hyde SC; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Gene Medicine Research Group, NDCLS, John Radcliffe Hospital, Oxford, UK., Innes JA; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Centre for Genomic and Experimental Medicine, IGMM, University of Edinburgh, Edinburgh, UK., Maher TM; Fibrosis Research Group, Inflammation, Repair & Development Section, National Heart and Lung Institute, Sir Alexander Fleming Building, Imperial College, London, UK., Moran C; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Meng C; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Paul-Smith MC; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Pringle IA; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Gene Medicine Research Group, NDCLS, John Radcliffe Hospital, Oxford, UK., Pytel KM; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Rodriguez-Martinez A; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Schmidt AC; Ave Leopold Wiener, Brussels, Belgium., Stevenson BJ; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Centre for Genomic and Experimental Medicine, IGMM, University of Edinburgh, Edinburgh, UK., Sumner-Jones SG; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK.; Gene Medicine Research Group, NDCLS, John Radcliffe Hospital, Oxford, UK., Toshner R; Fibrosis Research Group, Inflammation, Repair & Development Section, National Heart and Lung Institute, Sir Alexander Fleming Building, Imperial College, London, UK., Tsugumine S; ID Pharme Co. Ltd. (DNAVEC Center), Tsukuba, Japan., Wasowicz MW; Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, UK.; UK Cystic Fibrosis Gene Therapy Consortium, Oxford, UK., Zhu J; Lung Pathology Unit, Department of Airway Disease Infection, NHLI, Imperial College London, London, UK.

Publikováno v: Thorax [Thorax] 2017 Feb; Vol. 72 (2), pp. 137-147. Date of Electronic Publication: 2016 Nov 16.