Besides infection, CFTR-autophagy dysfunction can lead to an impaired clearance of cellular debris and products that would normally be degraded. One such product is ceramide, which in response to CFTR-autophagy dysfunction can accumulate in aggresome bodies within cells [22,110,182,183,184,185]. Ceramide accumulation has been observed in both CF patients with CFTR mutations [182,183,186] and COPD patients who have acquired CFTR dysfunction due to CS exposure [22,110].