The genesis of cystic fibrosis lung disease

JJ Wine - The Journal of clinical investigation, 1999 - Am Soc Clin Investig
James M. Wilson, Editor ductance, and by analogy with the sweat duct (11, 12) this allows
salt levels in the ASL to remain at levels similar to those in plasma. The high salt in the ASL …

The innate immune system in cystic fibrosis lung disease

R Bals, DJ Weiner, JM Wilson - The Journal of clinical …, 1999 - Am Soc Clin Investig
The story of cystic fibrosis (CF) research over the last decade has provided important
lessons about the power and limitations of positional cloning of disease genes, the …

Pulmonary epithelial sodium-channel dysfunction and excess airway liquid in pseudohypoaldosteronism

E Kerem, T Bistritzer, A Hanukoglu… - … England Journal of …, 1999 - Mass Medical Soc
Background Active sodium absorption is the dominant mechanism of ion transport in airway
epithelium, but its role in pulmonary physiology and airway host defense is unknown. To …

Innate antimicrobial activity of nasal secretions

AM Cole, P Dewan, T Ganz - Infection and immunity, 1999 - Am Soc Microbiol
Minimally manipulated nasal secretions, an accessible form of airway surface fluid, were
tested against indigenous and added bacteria by using CFU assays. Antimicrobial activity …

Neutrophil antibacterial peptides, multifunctional effector molecules in the mammalian immune system

GH Gudmundsson, B Agerberth - Journal of immunological methods, 1999 - Elsevier
The bactericidal machinery of mammalian neutrophils is built up of many components with
different chemical properties, involving proteins, peptides and oxygen-dependent radicals …

Molecular insights into the physiology of the 'thin film'of airway surface liquid

RC Boucher - The Journal of Physiology, 1999 - Wiley Online Library
The epithelia that line the airways of the lung exhibit two general functions:(1) airway
epithelia in all regions 'defend'the lung against infectious and noxious agents; and (2) …

The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: a pilot study

M Robinson, E Daviskas, S Eberl… - European …, 1999 - Eur Respiratory Soc
It has been postulated that hypertonic saline (HS) might impair the antimicrobial effects of
defensins within the airways. Alternative non-ionic osmotic agents such as mannitol may …

Cystic fibrosis transmembrane conductance regulator regulates luminal Cl−/HCO3− exchange in mouse submandibular and pancreatic ducts

MG Lee, JY Choi, X Luo, E Strickland… - Journal of Biological …, 1999 - ASBMB
We have demonstrated previously the regulation of Cl−/HCO 3− exchange activity by the
cystic fibrosis transmembrane conductance regulator (CFTR) in model systems of cells …

[HTML][HTML] Cystic fibrosis and the salt controversy

WB Guggino - Cell, 1999 - cell.com
The human airway has two aqueous layers that are vital for normal lung function. A mucous
layer traps inhaled bacteria and foreign particles. A thin layer of airway surface fluid (ASL) …

Cystic fibrosis: an inherited susceptibility to bacterial respiratory infections

B Tümmler, C Kiewitz - Molecular medicine today, 1999 - cell.com
Cystic fibrosis is a severe monogenic disorder of ion transport in exocrine glands. The basic
defect predisposes to chronic bacterial airway infections with Staphylococcus aureus …