Role of CIC-5 in the pathogenesis of hypercalciuria: recent insights from transgenic mouse models

Dent's disease is an inherited disorder characterized by hypercalciuria, lo w molecular weight proteinuria, and Fanconi syndrome, which is caused by in activating mutations in CIC-5, a chloride channel expressed in endosomes of the proximal renal tubule. The role of CIC-5 in the pathogenesis of the hy percalciuria and other myriad manifestations of this disease, however, is l argely unknown. New insights from three new transgenic mouse models of Dent 's disease, reported in the past year, are discussed. Curr Opin Nephrol Hyp ertens 10:415-420. (C) 2001 Lippincott Williams & Wilkins.