ADTKD is associated with at least five pathogenic variants: UMOD, MUC1, REN, HNF1B, and the rare SEC61A1. As of the end of November 2022, 35 reports of pathogenic variants in the REN gene are documented in the Global Variome shared LOVD database, with 16 identified disease-causing variants (7). Here, SEC61A1 is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.