To date, identified ADTKD-associated genes are UMOD (uromodulin, 16p12), MUC1 (mucin-1, 1q22), HNF1B (HNF1-beta, 17q12), REN (renin, 1q32) and SEC61A1 (alpha1-subunit of translocon 61, 3q21). Here, HNF1B is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.