Six mutations in the REN gene and more than 100 mutations in the UMOD gene have been identified that cause a progressive decline in kidney functionality and are diagnosed as ADTKD–REN and ADTKD–UMOD, respectively (Devuyst et al, 2019; Schaeffer et al, 2019; Olinger et al, 2020). Here, REN is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.