In double-immunostaining using anti-UMOD antibody and various organelle markers in an ADTKD-UMOD patient kidney (case #1), accumulated UMOD proteins were merged with increased ER proteins but not with Golgi, lysosome or secretory granules (Fig. 2), meaning that mutated UMOD proteins are captured in ER and cannot proceed further. Here, UMOD is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.