‘Autosomal dominant tubulointerstitial kidney disease – UMOD’ (ADTKD-UMOD; formerly uromodulin-associated kidney disease - UAKD) is a progressive dominant hereditary chronic kidney disease (CKD)1, 2 caused by amino acid changing mutations of the gene encoding the glycoprotein uromodulin (UMOD). The gene discussed is ART4; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.