Fig. 3.

Ceramide mediates lung fibrosis in cystic fibrosis mice. Fibrosis in the lung of 8 month old Cftr-deficient mice, Cftr-deficient mice heterozygous for acid sphingomyelinase, Cftr-deficient mice treated with amitriptyline or fluoxetine and wildtype mice was determined by measuring collagen using the Sircol Dye technique (A) and masson-trichrome staining (B). Data are the mean ± SD with n = 6 per group. *p < 0.001 compared as indicated. (B). Genetic or pharmacological inhibition of acid sphingomyelinase minimized lung fibrosis in Cftr-deficient mice. Representative lung sections from 6 mice per group are shown. Magnification is 400×.