These include Dent disease (CLCN5, OCRL), AD tubulointerstitial kidney disease (ADTKD due to UMOD variants), nephronophthisis (TTC21B, NPHP4), Imerslund-Grasbeck syndrome 1 (CUBN) and papillorenal syndrome (PAX2). The gene discussed is CUBN; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.