CLCN5 and Dent disease type 1: The human voltage-gated 2Cl−/H+ exchange transporter, ClC-5, is an epithelial membrane exchanger that facilitates protein and Ca2+ absorption in the kidney.1, , , , –6 The physiological role of ClC-5 is portrayed by the clinical characteristics of patients with Dent disease type 1 (DD1) who have a disease-causing variant in the X-chromosome gene, CLCN5.