ADTKD is a monogenic disease caused by mutations in five different genes, including UMOD, MUC1, REN, HNF1β, and SEC61A1 which encode uromodulin, transmembrane epithelial mucin 1, renin, hepatocyte nuclear factor 1β and α1 subunit of the SEC61, respectively [49]. Here, HNF1B is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.