Article
[Cystic fibrosis lung disease and its management].
Nihon rinsho. Japanese journal of clinical medicine - 1 Sept 1999
Hidaka N, Hidaka Y
Abstract excerpt
The function of the cystic fibrosis (CF) gene product, cystic fibrosis transmembrane conductance regulator (CFTR) is critical to normal lung defense. CFTR not only acts as a cAMP-dependent Cl-channel, but regulates other ion channels and several cellular functions. This article describes the current understanding of the clinical pathophysiology and the management of cystic fibrosis lung disease, particularly the...
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