CLCN5 and Dent disease: Dent disease 1 (OMIM phenotype number 300009) is an XLR condition resulting from inactivating variants in CLCN5. Hallmark features are progressive proximal renal tubulopathy with LMW proteinuria, hypercalciuria, NC, NL, and progressive ESKD (Claverie-Martín et al., 2011).