Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
OBJECTIVE
To determine whether there is an association between mutations of the cystic fibrosis transmembrane regulator (CFTR) and the predilection of patients with cystic fibrosis (CF) for Pseudomonas aeruginosa infection.


METHODS
We quantified the adherence of P. aeruginosa PA01, labeled with sulfur 35-methionine, to epithelial monolayers derived from nasal scrapings of patients with specific CFTR mutations, and of carriers and normal subjects.


RESULTS
Adherence of P. aeruginosa to epithelial cells from patients with CF was significantly greater than to cells from either carriers (t = 2.94; p = 0.009) or normal subjects (t = 3.32; p = 0.004). Adherence to epithelial cells from patients with CF who were homozygous for the delta F508 mutation ranged from 12% to 35% (mean, 23.7%) of the added inoculum, which was significantly greater than the binding to cells from patients with other mutations, which ranged from 3% to 18% (mean, 9.4%; t = 3.71; p = 0.002), from heterozygote carriers (3% to 11%; mean, 7.9%; t = 4.87; p = 0.002), or from normal subjects (2% to 10%: mean, 7.0%; t = 5.21; p = 0.002).


CONCLUSIONS
Adherence to P. aeruginosa can be correlated with homozygosity for the delta 508 mutation; CFTR dysfunction may be one of the factors involved in the pathogenesis of pulmonary infection in CF.
Zar
H
H
Department of Pediatrics, College of Physicians and Surgeons, Columbia University, New York, NY 10032.
Saiman
L
L
Quittell
L
L
Prince
A
A
D000293:Adolescent; D000328:Adult; D001422:Bacterial Adhesion; D002478:Cells, Cultured; D018118:Chloride Channels / Q000235:genetics*; D003550:Cystic Fibrosis / Q000235:genetics / Q000382:microbiology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D004198:Disease Susceptibility; D004847:Epithelial Cells; D004848:Epithelium / Q000382:microbiology; D005260:Female; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000235:genetics*; D008875:Middle Aged; D009154:Mutation; D011552:Pseudomonas Infections / Q000235:genetics / Q000382:microbiology; D011550:Pseudomonas aeruginosa / Q000472:pathogenicity*; D014420:Turbinates / Q000166:cytology / Q000382:microbiology*
C505032:CFTR protein, human; D018118:Chloride Channels; D008565:Membrane Proteins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator