Cystic fibrosis: A disorder of biological signal transduction

In this issue of the Journal an article by Wattchow et al. (from the laboratories of Prof. J. B. Furness in Adelaide) reports that vasoactive intestinal peptide (VIP)‐containing nerve terminals, normally present in the subepithelial regions of the nasal and intestinal mucosa, are absent in cystic fibrosis (CF) patients, although similar nerves supplying the enteric musculature are still present. Since nervous tissue is obviously not epithelial, these results draw attention to what is often overlooked, namely that, although CF manifests itself conspicuously as a disorder of epithelial transport function, it affects many non‐epithelial tissues as well. The appearance of Wattchow's elegant histochemical study provides us with a timely opportunity to review recent progress in the search for the underlying defect in CF. This brief review must necessarily be highly selective and the interested reader is referred to the reviews of Frizzell, Welsh and Fick and McPherson and Goodchild for additional reference.