Origins of cystic fibrosis lung disease

A Munder, B Tuemmler, DA Stoltz… - The New England …, 2015 - iro.uiowa.edu
Abstract To the Editor: On the basis of their seminal findings in models of disease in large
animals, Stoltz and colleagues (Jan. 22 issue) 1 report that the origins of cystic fibrosis lung
disease result from impaired chloride and bicarbonate epithelial transport, defective
mucociliary transport, and acidification of the airway-surface liquid. The pathophysiology of
cystic fibrosis is deduced from the loss or dysfunction of CFTR in the apical epithelial
membrane. However, this exclusive focus on the epithelial membrane ignores the roles of …

Origins of cystic fibrosis lung disease

DA Stoltz, DK Meyerholz, MJ Welsh - New England Journal of …, 2015 - Mass Medical Soc
Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator
undermine many host defense systems by inhibiting the function of airway-surface liquid,
causing flaws in mucociliary transport, and compromising other lung-protection
mechanisms.