HNF1B and autosomal dominant medullary cystic kidney disease with or without hyperuricemia: However, the R177Q mutation also caused ADTKD and MODY5, which suggest the importance of POU-containing domains in HNF1B. The proband's mother maintained mildly increased serum creatinine and bland urinary sediments 20 years after the onset of MODY, suggesting that the R177Q mutation may lead to a slow-progressive kidney phenotype.