Figure 3.

CFTR rescue by DAP in the CFTR-NS mouse model

(A) Proportions of the three genotypes among live newborns. Mothers were exposed to DMSO or DAP by daily PO exposure during gestation. The counts are from four mothers treated with DMSO and four mothers treated with DAP for one to five litters and the total number of newborn mice analyzed is indicated on the top of each bar. A statistical analysis resulted in a p value lower than 0.01, indicating a strong trust in these results (Monte-Carlo method). (B) CFTR (in red) in the intestine (duodenum), detected by IHC applied to tissue from WT and HO newborns, from breastfed pups whose mothers were exposed to DMSO or DAP by daily PO exposure during and after gestation, and from adults (WT and HO) exposed to 3 days of DMSO or DAP by daily PO exposure. (C) CFTR (in red) detected by IHC in lung (around the bronchi) and intestinal (duodenum) tissues from 2-week-breastfed WT and HO pups whose mothers were exposed PO daily to DMSO or DAP. All the IHCs are representative of at least five mice. (D) Measurement of the presence of DAP in the contents of the stomach of newborns. Each sample is a mix of the contents of the entire litter.