Mutations of the human gene encoding ClC-5 (CLCN5 gene) cause the X-linked disorder of the proximal tubules known as Dent’s disease, which is characterized by low-molecular-weight proteinuria, hypercalciuria, nephrolithiasis, aminoaciduria, phosphaturia, and renal failure [83,84]. The gene discussed is CLCN5; the disease is nephrolithiasis.