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F508 mice: role of
airway surface liquid composition
Departments of Pediatrics, Internal Medicine, and Occupational and Environmental Health, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242
Cystic fibrosis mice have been generated by gene
targeting but show little lung disease without repeated exposure to
bacteria. We asked if murine mucosal defenses and airway surface liquid (ASL) Cl
were altered by
the 
F508 cystic fibrosis transmembrane conductance regulator
mutation. Naive F508 
/ and +/ mice showed no
pulmonary inflammation and after inhaled Pseudomonas
aeruginosa had similar inflammatory responses and
bacterial clearance rates. We therefore investigated components of the
innate immune system. Bronchoalveolar lavage fluid from mice killed
Escherichia coli, and the microbicidal activity was inhibited by NaCl. Because 
-defensins are
salt-sensitive epithelial products, we looked for pulmonary
-defensin expression. A mouse homolog of human -defensin-1
(termed "MBD-1") was identified; the mRNA was expressed in the
lung. Using a radiotracer technique, ASL volume and
Cl concentration
([Cl]) were
measured in cultured tracheal epithelia from normal and F508
/ mice. The estimated ASL volume was similar for both groups. There were no differences in ASL
[Cl] in F508
/ and normal mice (13.8 ± 2.6 vs. 17.8 ± 5.6 meq/l). Because ASL
[Cl] is low in
normal and mutant mice, salt-sensitive antimicrobial factors, including
MBD-1, may be normally active.
cystic fibrosis transmembrane conductance regulator; Pseudomonas aeruginosa; defensin; cystic fibrosis
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