UMOD and autosomal dominant medullary cystic kidney disease with or without hyperuricemia: C125 in mouse uromodulin is the equivalent of C126 in human uromodulin, and mutation of this residue in humans to an arginine (C126R) has been reported to cause ADTKD-UMOD (Lhotta et al., 1998; Turner et al., 2003).