Fatty Acid Metabolism in Cystic Fibrosis

In 1989, when the gene locus for cystic fibrosis was identified, expectations were great for rapid progress in understanding and effectively treating the disease. It was generally believed that cystic fibrosis was a monogenic disease, which would possibly soon be treatable by gene therapy. Now, 15 years later, it has turned out that nature is much more complicated than we had predicted. For example, even though more than 1200 mutations have been identified in the cystic fibrosis transmembrane conductance regulator gene (CFTR), the search for a relation between the different phenotypes and mutations has mainly failed. Furthermore, siblings . . .