In our study, on the basis of the reduced renal mRNA expression of autophagy-related genes and increased expression of the p62 protein in cystic lining epithelial cells, our results confirmed the impairment of autophagy in Pkd1-deficient mice and supported a specific role of autophagy in the pathogenesis of ADPKD [44]. Here, SQSTM1 is linked to autosomal dominant polycystic kidney disease.