Both clinical and animal model studies suggest a compensatory role for CLCAs in the context of cystic fibrosis (CF): the fatal intestinal disease, meconium ileus, arising in CFTR-deficient mice is corrected by overexpression of mCLCA3 (an orthologue of human CLCA1) (Young et al., 2007) and, correspondingly, mutations in CLCA1 are found in a subset of CF patients with aggravated intestinal disease (van der Doef et al., 2010). Here, CLCA1 is linked to Meconium ileus.