A Review. Cystic Fibrosis at Forty—Quo Vadis?

Na and K dependence of the Na/K pump in cystic fibrosis fibroblasts.

“…The expected gene frequency is 1/20 (15,16). The clinical disease affects predominately exocrine glands, producing pancreatic and pulmonary insufficiency.…”

Aldosterone Metabolism and Transepithelial Potential Difference in Normal and Cystic Fibrosis Subjects

“…Because systematic assessment of the mucin component of respiratory "secretions" in CF patients has failed to identify a unique abnormality in the mucus glycoproteins (2), and because the secretions of the gut, reproductive tract, and airways of CF patients appear to have a reduced water content (3), we have recently explored abnormalities in the epithelial ion transport processes that regulates the salt and water content of respiratory secretions of CF patients.…”

Lysozyme Activity in Cystic Fibrosis

“…Sera from cystic fibrosis homozygotes and heterozygotes were found to promote lysosomal degradation of sensitized human leukocytes, as measured by enzyme release to a greater extent than normal sera (6). Considerable controversy has evolved from studies of cystic fibrosis fibroblasts and the multiple leakage of hydrolytic enzymes in that findings of some investigators have not been reproduced by others, possibly because of differences in technical methods (8). Although the studies on skin fibroblasts have been challenged (10,19,20), the concept of a "leaky lysosome" defect cannot be discounted.…”