‘Autosomal dominant tubulointerstitial kidney disease – UMOD’ (ADTKD-UMOD) is caused by impaired maturation and secretion of mutant uromodulin (UMOD) in thick ascending limb of Henle loop (TAL) cells, resulting in endoplasmic reticulum (ER) stress and unfolded protein response (UPR). The gene discussed is UMOD; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.