Defective autophagy allows B. cepacia to survive and replicate in ΔF508 macrophages, of which the CFR F508 mutation is the most common in cystic fibrosis patients.37 In macrophages where p62 is elevated, other cell components clump together, causing disruption to the autophagy process. This evidence concerns the gene SQSTM1 and cystic fibrosis.