Given that (i) the expression of REN and UMOD is almost exclusively restricted to cells of the nephron and (ii) these proteins are involved in renal function, mass, or development we propose that the known cases of ADTKD–SEC61A1 phenocopy to a large extent the clinical hallmarks of ADTKD–REN and, in case of V67G, ADTKD–UMOD (Gribouval et al, 2005; Castrop et al, 2010; Gomez & Sequeira-Lopez, 2016; Steubl et al, 2016; Pivin et al, 2018). Here, UMOD is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.