These researchers demonstrated in previous projects (FFC#21/2010, FFC#16/2012, FFC#22/2014) that blocking IL-1βsignaling with the IL-1 receptor antagonist, anakinra, successfully ameliorated pathogenic CF inflammation. Now, preliminary data suggested that anakinra increased cell surface expression of F508del-CFTR and chloride transport in CFBE41o-cells and HBE cells from CF patients. Thus they aim to evaluate the effects of anakinra on maturation, stability and function of F508del-CFTR . Studies will be carried out in vitro on HBE cells from F508del-CFTR patients and controls and in vivo in F508del mutant mice either testing anakinra alone or in combination with ivacaftor and lumacaftor.