Interestingly, a recent study reported that GLIS3 expression was decreased in an early-onset autosomal dominant polycystic kidney disease (ADPKD) model [50], consistent with the concept that down-regulation of GLIS3 may play a role in ADPKD development and/or progression similar to Glis3-deficiency, thereby increasing the significance of our findings to the study of renal cystic disease. Here, GLIS3 is linked to autosomal dominant polycystic kidney disease.