Alveolar inflammation in cystic fibrosis.

[1]  L. Taussig,et al.  Quantitative aspects of lung pathology in cystic fibrosis. , 2015, The American review of respiratory disease.

[2]  N. Høiby,et al.  Pseudomonas aeruginosa biofilms in the respiratory tract of cystic fibrosis patients , 2009, Pediatric pulmonology.

[3]  M. Weller,et al.  Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis , 2008, Nature Medicine.

[4]  E. Naline,et al.  Expression of cystic fibrosis transmembrane conductance regulator in the human distal lung. , 2008, Human pathology.

[5]  A. Nicholson,et al.  Airway remodelling in children with cystic fibrosis , 2007, Thorax.

[6]  V. Bindokas,et al.  CFTR regulates phagosome acidification in macrophages and alters bactericidal activity , 2006, Nature Cell Biology.

[7]  T. Machen.  Innate immune response in CF airway epithelia: hyperinflammatory? , 2006, American journal of physiology. Cell physiology.

[8]  M. Berger,et al.  Acute Pseudomonas challenge in cystic fibrosis mice causes prolonged nuclear factor-kappa B activation, cytokine secretion, and persistent lung inflammation. , 2006, The Journal of allergy and clinical immunology.

[9]  N. Pedemonte,et al.  Erratum: Contribution of CFTR to apical-basolateral fluid transport in cultured human alveolar epithelial type II cells (American Journal of Physiology - Lung Cellular and Molecular Physiology (February 2006) 290, 34, (L242-L249)) , 2006 .

[10]  L. Jain,et al.  Functional ion channels in pulmonary alveolar type I cells support a role for type I cells in lung ion transport. , 2006, Proceedings of the National Academy of Sciences of the United States of America.

[11]  N. Pedemonte,et al.  Contribution of CFTR to apical-basolateral fluid transport in cultured human alveolar epithelial type II cells. , 2006, American journal of physiology. Lung cellular and molecular physiology.

[12]  J. Riordan,et al.  Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia. , 2005, Molecular biology of the cell.

[13]  I. Petrache,et al.  Ceramide upregulation causes pulmonary cell apoptosis and emphysema-like disease in mice , 2005, Nature Medicine.

[14]  D. Look,et al.  NF-kappaB activation and sustained IL-8 gene expression in primary cultures of cystic fibrosis airway epithelial cells stimulated with Pseudomonas aeruginosa. , 2005, American journal of physiology. Lung cellular and molecular physiology.

[15]  S. Randell,et al.  Cytokine secretion by cystic fibrosis airway epithelial cells. , 2004, American journal of respiratory and critical care medicine.

[16]  H. Chapman.  Disorders of lung matrix remodeling. , 2004, The Journal of clinical investigation.

[17]  R. Boucher,et al.  New concepts of the pathogenesis of cystic fibrosis lung disease , 2004, European Respiratory Journal.

[18]  S. Manna,et al.  Oleandrin suppresses activation of nuclear transcription factor-kappa B and activator protein-1 and potentiates apoptosis induced by ceramide. , 2003, Biochemical pharmacology.

[19]  R. Levine,et al.  Inactivation of Human β-Defensins 2 and 3 by Elastolytic Cathepsins1 , 2003, The Journal of Immunology.

[20]  A. Bush,et al.  Growth factors in cystic fibrosis - when more is not enough. , 2003, Paediatric respiratory reviews.

[21]  F. Ratjen,et al.  Cystic fibrosis , 2003, The Lancet.

[22]  R. Kolesnick,et al.  Host defense against Pseudomonas aeruginosa requires ceramide-rich membrane rafts , 2003, Nature Medicine.

[23]  F. Ratjen,et al.  Matrix metalloproteases in BAL fluid of patients with cystic fibrosis and their modulation by treatment with dornase alpha , 2002, Thorax.

[24]  J. Hogg,et al.  Chronic obstructive pulmonary disease - part 2: pathology and biochemistry of emphysema. , 2002, Thorax.

[25]  R. Tirouvanziam,et al.  Primary inflammation in human cystic fibrosis small airways. , 2002, American journal of physiology. Lung cellular and molecular physiology.

[26]  S. Monti,et al.  Urinary desmosine excretion is inversely correlated with the extent of emphysema in patients with chronic obstructive pulmonary disease. , 2002, The international journal of biochemistry & cell biology.

[27]  T. Tetley.  Macrophages and the pathogenesis of COPD. , 2002, Chest.

[28]  V. Starnes,et al.  Clinical findings and lung pathology in children with cystic fibrosis. , 2002, American journal of respiratory and critical care medicine.

[29]  C. Thompson,et al.  Activation and inhibition of lymphocytes by costimulation. , 2002, The Journal of clinical investigation.

[30]  J. Emerson,et al.  Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis * , 2001, Pediatric pulmonology.

[31]  R. Bryan,et al.  Activation of NF-kappaB in airway epithelial cells is dependent on CFTR trafficking and Cl- channel function. , 2001, American journal of physiology. Lung cellular and molecular physiology.

[32]  B Cao,et al.  The potential role of PDGF, IGF-1, TGF-beta expression in idiopathic pulmonary fibrosis. , 2000, Chinese medical journal.

[33]  R. Stockley,et al.  MEKC of desmosine and isodesmosine in urine of chronic destructive lung disease patients. , 2000, The European respiratory journal.

[34]  W. Hop,et al.  Cartilaginous airway wall dimensions and airway resistance in cystic fibrosis lungs. , 2000, The European respiratory journal.

[35]  Y. Hannun,et al.  Signal transduction and the regulation of apoptosis: roles of ceramide , 1998, Apoptosis.

[36]  I. Durieu,et al.  Subepithelial fibrosis and degradation of the bronchial extracellular matrix in cystic fibrosis. , 1998, American journal of respiratory and critical care medicine.

[37]  E. M. Laursen,et al.  Controlled trial of inhaled budesonide in patients with cystic fibrosis and chronic bronchopulmonary Psuedomonas aeruginosa infection. , 1997, American journal of respiratory and critical care medicine.

[38]  N. Klein,et al.  Randomised controlled trial of inhaled corticosteroids (fluticasone propionate) in cystic fibrosis , 1997, Archives of disease in childhood.

[39]  C. Delacourt,et al.  Imbalance between 95 kDa type IV collagenase and tissue inhibitor of metalloproteinases in sputum of patients with cystic fibrosis. , 1995, American journal of respiratory and critical care medicine.

[40]  M. Weinberger,et al.  The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis , 1995, Pediatric pulmonology.

[41]  M. Konstan,et al.  Elastin and collagen degradation products in urine of patients with cystic fibrosis. , 1995, American journal of respiratory and critical care medicine.

[42]  D. Riches,et al.  Early pulmonary inflammation in infants with cystic fibrosis. , 1995, American journal of respiratory and critical care medicine.

[43]  J. Hayes,et al.  Neutrophil collagenase in sputum from patients with cystic fibrosis. , 1994, American journal of respiratory and critical care medicine.

[44]  R. D. du Bois,et al.  Insulin-like growth factor-I is partially responsible for fibroblast proliferation induced by bronchoalveolar lavage fluid from patients with systemic sclerosis. , 1994, Clinical science.

[45]  R. Kolesnick,et al.  Tumor necrosis factor activation of the sphingomyelin pathway signals nuclear factor kappa B translocation in intact HL-60 cells. , 1993, The Journal of biological chemistry.

[46]  R. Crystal,et al.  Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. , 1992, The Journal of clinical investigation.

[47]  C. Wohlford-Lenane,et al.  Localization of cystic fibrosis transmembrane conductance regulator mRNA in human fetal lung tissue by in situ hybridization. , 1992, The Journal of clinical investigation.

[48]  R. Crystal,et al.  Aerosol α1 -antitrypsin treatment for cystic fibrosis , 1991, The Lancet.

[49]  L. Tsui,et al.  Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. , 1989, Science.

[50]  L. Tsui,et al.  Identification of the cystic fibrosis gene: genetic analysis. , 1989, Science.

[51]  P. Pilch,et al.  Stimulation of collagen formation by insulin and insulin-like growth factor I in cultures of human lung fibroblasts. , 1989, Endocrinology.

[52]  G. Döring,et al.  Lysosomal enzymes from polymorphonuclear leukocytes and proteinase inhibitors in patients with cystic fibrosis. , 1986, The American review of respiratory disease.

[53]  S. Greenberg,et al.  The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. , 1976, Human pathology.

[54]  J. Riordan,et al.  Identification of the Cystic Fibrosis Gene : Chromosome Walking and Jumping Author ( s ) : , 2008 .

[55]  D. Dusser,et al.  Relationship between IκBα deficiency, NFκB activity and interleukin-8 production in CF human airway epithelial cells , 2001, Pflügers Archiv.

[56]  R. Boucher,et al.  Pathophysiology of gene-targeted mouse models for cystic fibrosis. , 1999, Physiological reviews.

[57]  J. Warner,et al.  Immunology of cystic fibrosis. , 1992, British medical bulletin.