Papers 24

  1. Cystic fibrosis: to ion transport and beyond.
    Bush A et al., Eur Respir J. 2010 Nov;36(5):991-2. doi: 10.1183/09031936.00056310.
  2. Pseudomonas aeruginosa infection in cystic fibrosis: pathophysiological mechanisms and therapeutic approaches.
    Lund-Palau H et al., Expert Rev Respir Med. 2016 Jun;10(6):685-97. doi: 10.1080/17476348.2016.1177460. Epub 2016 May 13.
  3. Cystic fibrosis.
    Davies JC et al., BMJ. 2007 Dec 15;335(7632):1255-9.
  4. Bone marrow stem cells do not repopulate the healthy upper respiratory tract.
    Davies JC et al., Pediatr Pulmonol. 2002 Oct;34(4):251-6.
  5. Mannose-binding lectin is present in the infected airway: a possible pulmonary defence mechanism.
    Fidler KJ et al., Thorax. 2009 Feb;64(2):150-5. doi: 10.1136/thx.2008.100073. Epub 2008 Nov 6.
  6. Reduction in the adherence of Pseudomonas aeruginosa to native cystic fibrosis epithelium with anti-asialoGM1 antibody and neuraminidase inhibition.
    Davies JC et al., Eur Respir J. 1999 Mar;13(3):565-70.
  7. Airway surface pH in subjects with cystic fibrosis.
    McShane D et al., Eur Respir J. 2003 Jan;21(1):37-42.
  8. Normal nasal mucociliary clearance in CF children: evidence against a CFTR-related defect.
    McShane D et al., Eur Respir J. 2004 Jul;24(1):95-100.
  9. Airway remodelling and its relationship to inflammation in cystic fibrosis.
    Regamey N et al., Thorax. 2011 Jul;66(7):624-9. doi: 10.1136/thx.2009.134106. Epub 2010 Oct 1.
  10. Lung clearance index in primary ciliary dyskinesia and bronchiectasis.
    Irving SJ et al., Am J Respir Crit Care Med. 2014 May 1;189(9):1147-8. doi: 10.1164/rccm.201402-0206LE.
  11. Does mass spectrometric breath analysis detect Pseudomonas aeruginosa in cystic fibrosis?
    Pabary R et al., Eur Respir J. 2016 Mar;47(3):994-7. doi: 10.1183/13993003.00944-2015. Epub 2016 Feb 4.
  12. Exploring the mechanisms of macrolides in cystic fibrosis.
    Equi AC et al., Respir Med. 2006 Apr;100(4):687-97. Epub 2005 Sep 26.
  13. Airway remodelling in children with cystic fibrosis.
    Hilliard TN et al., Thorax. 2007 Dec;62(12):1074-80. Epub 2007 May 25.
  14. Quantification of periciliary fluid height in human airway biopsies is feasible, but not suitable as a biomarker.
    Griesenbach U et al., Am J Respir Cell Mol Biol. 2011 Mar;44(3):309-15. doi: 10.1165/rcmb.2009-0265OC. Epub 2010 Apr 23.
  15. Biomarkers for cystic fibrosis lung disease: application of SELDI-TOF mass spectrometry to BAL fluid.
    MacGregor G et al., J Cyst Fibros. 2008 Sep;7(5):352-8. doi: 10.1016/j.jcf.2007.12.005. Epub 2008 Feb 1.
  16. Multiple breath washouts in children can be shortened without compromising quality.
    Ahmad F et al., Eur Respir J. 2015 Dec;46(6):1814-6. doi: 10.1183/13993003.00791-2015. Epub 2015 Oct 9.
  17. Nasal abnormalities in cystic fibrosis mice independent of infection and inflammation.
    Hilliard TN et al., Am J Respir Cell Mol Biol. 2008 Jul;39(1):19-25. doi: 10.1165/rcmb.2007-0284OC. Epub 2008 Jan 31.
  18. Endobronchial biopsy in childhood.
    Regamey N et al., Chest. 2008 Jan;133(1):312; author reply 313. doi: 10.1378/chest.07-1735.
  19. CFTR gene transfer reduces the binding of Pseudomonas aeruginosa to cystic fibrosis respiratory epithelium.
    Davies JC et al., Am J Respir Cell Mol Biol. 1997 Jun;16(6):657-63.
  20. Increased airway smooth muscle mass in children with asthma, cystic fibrosis, and non-cystic fibrosis bronchiectasis.
    Regamey N et al., Am J Respir Crit Care Med. 2008 Apr 15;177(8):837-43. doi: 10.1164/rccm.200707-977OC. Epub 2008 Jan 24.
  21. Potential difference measurements in the lower airway of children with and without cystic fibrosis.
    Davies JC et al., Am J Respir Crit Care Med. 2005 May 1;171(9):1015-9. Epub 2005 Jan 7.
  22. Quality, size, and composition of pediatric endobronchial biopsies in cystic fibrosis.
    Regamey N et al., Chest. 2007 Jun;131(6):1710-7. Epub 2007 Feb 22.
  23. Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis.
    Regamey N et al., Thorax. 2012 Feb;67(2):164-70. doi: 10.1136/thoraxjnl-2011-200585. Epub 2011 Oct 18.
  24. Lung clearance index and high-resolution computed tomography scores in primary ciliary dyskinesia.
    Irving SJ et al., Am J Respir Crit Care Med. 2013 Sep 1;188(5):545-9. doi: 10.1164/rccm.201304-0800OC.

 

DNA fragments being cut from an agarose gel exposed to UV.

 

E.coli from a large scale industrial production of our clinical trial plasmid pGM169.

 

Purifying mRNA from tissue samples.

 

Proposed 3D model of the CFTR protein.