The genesis of cystic fibrosis lung disease

作者：Jeffrey J. Wine · 发表于：Journal of Clinical Investigation · 年份：1999 · DOI：10.1172/jci6222 · 被引用次数：279 · 研究领域：Cystic Fibrosis Research Advances、Neonatal Respiratory Health Research、Tracheal and airway disorders

Cystic fibrosis (CF), as Bals et al. discuss in the other Perspective in this issue (1), is one of the most thoroughly understood genetic diseases. Thus it is frustrating that CF lung disease has been refractory to the onslaught of molecular/cellular research that has clarified so many other aspects of CF. But after many false starts, recent experiments are finally beginning to crack the shell of ignorance that surrounds CF lung disease. A series of innovative experiments, mainly from two laboratories, has revealed fascinating new aspects of normal and CF lung biology. Yet despite the care and resourcefulness of each group, their findings generate sharply contrasting views of CF lung disease that lead to opposite treatment strategies! Fortunately, this controversy is generating more light than heat and is illuminating the field to the ultimate benefit of CF clinical care.