SQSTM1 and cystic fibrosis: Specifically, it is defective in CF airways because of the depletion of the autophagy-related protein Beclin 1 (BECN1) with consequent accumulation of SQSTM1/p62 substrate which promotes a pro-inflammatory status sequestering misfolded ubiquitinated F508del-CFTR and anti-inflammatory proteins, such as PPARγ and IK-Bα.