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Role of the Cystic Fibrosis Transmembrane Conductance Regulator in Innate Immunity to Pseudomonas aeruginosa Infections
Pages 71-77

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From page 71...
... aeruginosa by inclusion in the bacterial inoculum of either free bacterial lipopolysaccharide or CFTR peptide 108-117 resulted in increased bacterial counts in the lungs. CFTR is also a receptor on gastrointestinal epithelial cells for Salmonella enterica serovar Typhi, the etiologic agent of typhoid fever.
From page 72...
... Thus, the molecular and cellular connections between lung infection and defects in CFTR have been of great interest as the primary determinant of the overall clinical status of patients with CF. Microbial Aspects of Lung Infection in CF Despite a complex sputum bacteriology, the progressive decline in pulmonary function that is the hallmark of CF is mostly attributable to a single pathogen, mucoid P
From page 73...
... aerug~nosa adherence to cells has been observed only when using primary cultures of nasal polyp cells from patients with CF homozygous for the Z\F508 CFTR allele when adherence is compared with that seen with primary respiratory epithelial cell cultures from healthy patients without CF and heterozygous carriers of mutant CFTR alleles (30, 35~. Cultured nasal polyp cells from patients with CF who are homozygous for other mutant CFTR alleles did not bind P
From page 74...
... Lung Epithelial Cellular Internalization of P aeruginosa Requires CFTR Binding to Bacterial LPS Core Oligosaccharide Internalization by epithelial cells may be a mechanism for clearing bacteria from the lung via cellular desquamation of internalized organisms.
From page 75...
... Airway epithelial cell uptake of P aeruginosa in the respiratory tract of a mammal was first described in a neonatal mouse model of lung infection by Prince and colleagues (47~.
From page 76...
... (B) Inhibition of translocation of serovar Typhi strain Ty2 from the Gl lumen of BALB/c mice infected with this bacterium plus a synthetic peptide corresponding to the indicated amino acids in the first predicted extracellular domain of CFTR.
From page 77...
... aerug~nosa and coordinates the epithelial cell response leading to bacterial clearance indicates a direct connection between mutant CFTR genes and the clinical course of CF. Thus, in addition to its 6 1.


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