Journal of Applied Physiology, Vol 70, Issue 5 2331-2333, Copyright © 1991 by American Physiological Society

ARTICLES

Molecular and cell biology of cystic fibrosis

P. B. Davis
Case Western Reserve University, Cleveland, Ohio 44106.

The questions emerged in better focus: we need to know, definitively, what CFTR is and what it does. We need to know how mutant CFTR expression leads to the relentless lung disease that takes the lives of the patients. We need to know how the different mutations in CFTR behave functionally. Much more information is needed on the pathways for ion transport in the airways in order for us to consider therapeutic alternatives. Better information on CFTR expression, particularly in the lung, would greatly facilitate consideration of pathophysiology as well as gene therapy. Many of these questions can be attacked by imaginative use of the tools already in hand. The need is urgent. The wondrous scientific advancements of the last five years and the additional money being spent on CF research have bought no dramatic increase in life expectancy for the patients. Every day, three more succumb.