Article
Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR.
American journal of respiratory and critical care medicine - 1 May 1997
Walker L C, Venglarik C J, Aubin G, Weatherly M R, McCarty N A, Lesnick B, Ruiz F, Clancy J P, Sorscher E J
Abstract excerpt
Patients with cystic fibrosis (CF) display defects in airway ion transport, but the influence of airway transport phenotype on improved prognosis is not known. We studied airway bioelectric properties in five CF patients with the rare A455E mutation that is associated with mild pulmonary disease. We also evaluated five patients possessing premature truncation mutations (G542X and R553X) for which an association...
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