Given the functional studies performed so far on mutations in the different ADTKD genes (i.e. UMOD, MUC1, SEC61A1 and REN), ER stress and unfolded protein response have been proposed to play a central role in disease onset and progression (Devuyst et al., 2019; Mabillard et al., 2023; Schaeffer and Olinger, 2020). The gene discussed is SEC61A1; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.