Embryonic lung growth is normal in a cftr-knockout mouse model.

The role of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel in embryonic lung growth remains uncertain. The authors used an established embryonic lung culture model to investigate the impact of cftr knockout on lung growth, airway peristalsis, and airway smooth muscle (ASM) distribution. Lung area, perimeter, lung bud count, and frequency of contraction were similar in wild-type (cftr +/+) and cftr knockout mice (cftr -/-). The percentage of mitotic cells was also consistent between genotypes in mesenchyme and epithelium. Smooth muscle distribution surrounding the airway appeared normally distributed in all genotypes. These data suggest that normal embryonic lung growth, ASM differentiation and airway peristalsis are CFTR independent.