An overview of the pathogenesis of cystic fibrosis lung disease.

[1]  J. Lo-Guidice,et al.  Human airway mucin glycosylation: A combinatory of carbohydrate determinants which vary in cystic fibrosis , 2001, Glycoconjugate Journal.

[2]  C. Scriver,et al.  The Metabolic and Molecular Bases of Inherited Disease, 8th Edition 2001 , 2001, Journal of Inherited Metabolic Disease.

[3]  R. Boucher,et al.  In vivo microdialysis for determination of nasal liquid ion composition. , 2002, American journal of physiology. Cell physiology.

[4]  R. Henry,et al.  Inflammation, infection, and pulmonary function in infants and young children with cystic fibrosis. , 2002, American journal of respiratory and critical care medicine.

[5]  M. Berger Lung inflammation early in cystic fibrosis: bugs are indicted, but the defense is guilty. , 2002, American journal of respiratory and critical care medicine.

[6]  T. Noah,et al.  Endotoxin activity and inflammatory markers in the airways of young patients with cystic fibrosis. , 2002, American journal of respiratory and critical care medicine.

[7]  J. Chao,et al.  Regulation of the Epithelial Sodium Channel by Serine Proteases in Human Airways* , 2002, The Journal of Biological Chemistry.

[8]  M. Knowles,et al.  Mucus clearance as a primary innate defense mechanism for mammalian airways. , 2002, The Journal of clinical investigation.

[9]  Richard C Boucher,et al.  Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients. , 2002, The Journal of clinical investigation.

[10]  M. Knowles,et al.  In Vivo Airway Surface Liquid Cl− Analysis with Solid-State Electrodes , 2002, The Journal of general physiology.

[11]  B. Tümmler,et al.  Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings. , 2001, The Journal of clinical investigation.

[12]  J. Gatzy,et al.  The Relative Roles of Passive Surface Forces and Active Ion Transport in the Modulation of Airway Surface Liquid Volume and Composition , 2001, The Journal of general physiology.

[13]  R. Tarran,et al.  The CF salt controversy: in vivo observations and therapeutic approaches. , 2001, Molecular cell.

[14]  J. Pilewski,et al.  Na+ transport in normal and CF human bronchial epithelial cells is inhibited by BAY 39-9437. , 2001, American journal of physiology. Lung cellular and molecular physiology.

[15]  A. Verkman,et al.  Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH. , 2001, The Journal of clinical investigation.

[16]  L. Sharples,et al.  Association of α1-antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis , 2001, Thorax.

[17]  R. Boucher,et al.  Expression and localization of epithelial aquaporins in the adult human lung. , 2001, American journal of respiratory cell and molecular biology.

[18]  M. Knowles,et al.  Prolonged airway retention of insoluble particles in cystic fibrosis versus primary ciliary dyskinesia. , 2000, Experimental lung research.

[19]  B. Tümmler,et al.  Cystic fibrosis: an inherited susceptibility to bacterial respiratory infections. , 1999, Molecular medicine today.

[20]  M. Knowles,et al.  Pulmonary epithelial sodium-channel dysfunction and excess airway liquid in pseudohypoaldosteronism. , 1999, The New England journal of medicine.

[21]  T. Ganz,et al.  Innate Antimicrobial Activity of Nasal Secretions , 1999, Infection and Immunity.

[22]  M. Corey,et al.  Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13 , 1999, Nature Genetics.

[23]  B. Tümmler,et al.  ΔF508 CFTR protein expression in tissues from patients with cystic fibrosis , 1999 .

[24]  R. C. Boucher,et al.  Molecular insights into the physiology of the ‘thin film’ of airway surface liquid , 1999, The Journal of physiology.

[25]  W. Guggino Cystic Fibrosis and the Salt Controversy , 1999, Cell.

[26]  J. Wine The genesis of cystic fibrosis lung disease. , 1999, The Journal of clinical investigation.

[27]  S. Randell,et al.  Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways Disease , 1998, Cell.

[28]  J. Riordan,et al.  Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome , 1998, The EMBO journal.

[29]  S. Randell,et al.  Coordinated clearance of periciliary liquid and mucus from airway surfaces. , 1998, The Journal of clinical investigation.

[30]  M. Welsh,et al.  Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro. , 1998, Molecular cell.

[31]  J. Hull,et al.  Elemental content of airway surface liquid from infants with cystic fibrosis. , 1998, American journal of respiratory and critical care medicine.

[32]  M. Knowles,et al.  Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects. , 1997, The Journal of clinical investigation.

[33]  B. Rossier,et al.  An epithelial serine protease activates the amiloride-sensitive sodium channel , 1997, Nature.

[34]  James M. Wilson,et al.  Human β-Defensin-1 Is a Salt-Sensitive Antibiotic in Lung That Is Inactivated in Cystic Fibrosis , 1997, Cell.

[35]  A. Wanner,et al.  Mucociliary clearance in the airways. , 1996, American journal of respiratory and critical care medicine.

[36]  E. Greenberg,et al.  Cystic Fibrosis Airway Epithelia Fail to Kill Bacteria Because of Abnormal Airway Surface Fluid , 1996, Cell.

[37]  J. Goldberg,et al.  Role of Mutant CFTR in Hypersusceptibility of Cystic Fibrosis Patients to Lung Infections , 1996, Science.

[38]  Satoshi Omura,et al.  Degradation of CFTR by the ubiquitin-proteasome pathway , 1995, Cell.

[39]  J C Olsen,et al.  CFTR as a cAMP-dependent regulator of sodium channels , 1995, Science.

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

[41]  A. Prince,et al.  Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. , 1995, Proceedings of the National Academy of Sciences of the United States of America.

[42]  J. Wilson,et al.  Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells. , 1995, The Journal of clinical investigation.

[43]  J. Zieleński,et al.  Cystic fibrosis: genotypic and phenotypic variations. , 1995, Annual review of genetics.

[44]  G. Cutting Genotype Defect: Its Effect on Cellular Function and Phenotypic Expression , 1994 .

[45]  R. Boucher,et al.  Human airway ion transport. Part two. , 1994, American Journal of Respiratory and Critical Care Medicine.

[46]  J. Yankaskas,et al.  Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: a patch-clamp study. , 1994, The American journal of physiology.

[47]  P. Quinton,et al.  Elemental composition of human airway surface fluid in healthy and diseased airways. , 1993, The American review of respiratory disease.

[48]  J. Widdicombe,et al.  Altered fluid transport across airway epithelium in cystic fibrosis. , 1993, Science.

[49]  J. Yankaskas,et al.  Sodium-permeable channels in the apical membrane of human nasal epithelial cells. , 1993, The American journal of physiology.

[50]  M. Welsh,et al.  Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis , 1993, Cell.

[51]  L. Tsui,et al.  The spectrum of cystic fibrosis mutations. , 1992, Trends in genetics : TIG.

[52]  J. Riordan,et al.  Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR) , 1992, Cell.

[53]  F. Collins,et al.  Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes. , 1991, Science.

[54]  R. Boucher,et al.  Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium. , 1991, The American journal of physiology.

[55]  R. Boucher,et al.  Sodium transport and intracellular sodium activity in cultured human nasal epithelium. , 1991, The American journal of physiology.

[56]  D Markiewicz,et al.  The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508). , 1990, The New England journal of medicine.

[57]  J. Marshall,et al.  Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis , 1990, Cell.

[58]  Matthew P. Anderson,et al.  Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells , 1990, Nature.

[59]  R. Boucher,et al.  Activation of an apical Cl- conductance by Ca2+ ionophores in cystic fibrosis airway epithelia. , 1989, The American journal of physiology.

[60]  M. Knowles,et al.  Oxygen Consumption and Ouabain Binding Sites in Cystic Fibrosis Nasal Epithelium , 1986, Pediatric Research.

[61]  L. Cantley,et al.  Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. , 1986, The Journal of clinical investigation.

[62]  M. Knowles,et al.  Abnormal ion permeation through cystic fibrosis respiratory epithelium. , 1983, Science.

[63]  P. Quinton,et al.  Chloride impermeability in cystic fibrosis , 1983, Nature.

[64]  M. Knowles,et al.  Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. , 1981, The New England journal of medicine.

[65]  I. Andersen,et al.  Clearance of inhaled particles from the human nose. , 1973, Archives of internal medicine.

[66]  K. Kilburn A hypothesis for pulmonary clearance and its implications. , 1968, The American review of respiratory disease.