Lack of Cftr in CD3+ Lymphocytes Leads to Aberrant Cytokine Secretion and Hyper-inflammatory Adaptive Immune Responses

Cystic fibrosis (CF), the most common fatal monogenic disease in the US, results from mutations in CFTR, a chloride channel. The mechanisms by which CFTR mutations cause lung disease in CF are not fully defined, but may include altered ion and water transport across the airway epithelium and aberrant inflammatory and immune responses to pathogens within the airways. We have shown that Cftr-/- mice mount an exaggerated IgE response towards Aspergillus fumigatus (Af) with higher levels of IL-13 and IL-4, mimicking both the Th-2 biased immune responses