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Genetic heterozygosity or functional inhibition of acid sphingomyelinase normalizes ceramide in lungs of cystic fibrosis mice. Ceramide was measured in lung extracts of wildtype, Cftr-deficient mice, Cftr-deficient mice heterozygous for acid sphingomyelinase or Cftr-deficient mice treated for 6.5 months with amitriptyline or fluoxetine. Ceramide was analyzed by a ceramide kinase assay. Data are the mean ± SD with n = 5 per group, ***p < 0.001 as indicated.
