The CLCN5 gene encodes a voltage-gated chloride ion channel, and mutations in this gene have been associated with several diseases all characterized by progressive proximal renal tubulopathy including Dent disease [MIM: 300009], X-linked recessive nephrolithiasis [MIM: 310468], X-linked recessive hypophosphatemia rickets [MIM: 300554], and low molecular weight proteinuria [MIM: 308990] [32–34]. The gene discussed is CLCN5; the disease is Dent disease.