Immunohistochemical localization of cystic fibrosis transmembrane conductance regulator in human fetal airway and digestive mucosa.

The cellular distribution of the cystic fibrosis transmembrane conductance regulator (CFTR) in human fetal digestive and respiratory mucosa has been studied by immunohistochemistry. The streptavidin-biotin immunoperoxidase method was applied to paraffin-embedded specimens collected from normal fetuses ranging from 7 to 39 wk of gestation. By the 7th wk, CFTR protein was strongly detected in the yolk sack; in contrast, the staining was weak in the undifferentiated epithelium of the intestine and the airways. At 12 wk, the intestine showed strongly and diffusely stained enterocytes and a basal cytoplasmic reactivity in the first secretory cells. During development, only slight changes could be detected in the digestive epithelial distribution of CFTR. In the airways, the CFTR distribution followed the cephalocaudal maturation. In the tracheal ciliated cells, the CFTR protein was diffusely detected in the cytoplasm as early as 7 wk. After 24-25 wk, CFTR was localized at the apical domain of the ciliated cells and was also present in the collecting ducts and in the glands of the airways, predominantly in the periphery of the acini. Our data suggest that the CFTR is present as early as 7 wk during organogenesis and probably plays an important role during fetal life. There is an evolution in the CFTR distribution during airway development, whereas, in the intestine, CFTR is highly expressed through the epithelium as early as 12 wk and keeps the same distribution until birth.