Function of CFTR Protein: Developmental Role, in Cystic fibrosis in the 21st century (2006).

Gill DR, Larson JE


In the lung, CFTR is largely expressed in the undifferentiated multipotent stem cells of the first- and second-trimester airway epithelium. As differentiation proceeds, the expression of CFTR dissipates and the adult lung expresses a small fraction of what was expressed in the fetus. There is growing evidence that infants with CF have lungs that are functionally and structurally abnormal prior to the appearance of clinical infection. This chapter examines the expression and role of CFTR in developmental cascades in the lung.