One of the earliest defects we observed following Myh9&10 inactivation was the accumulation of UMOD inside the TAL epithelium, which led us to compare the observed phenotype with the previously described ADTKD caused by mutations in UMOD (ADTKD-UMOD) (26, 27, 53). Here, UMOD is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.