Inactivating mutations of ClC-Kb lead to the inactivation of NKCC2 and ROMK channel and cause Bartter syndrome, a rare heterogeneous autosomal recessive nephropathy, characterized by reduced salt reabsorption in the kidney thick ascending limb of Henle, resulting in renal salt wasting and hypokalemic metabolic alkalosis (Konrad et al., 2000). Here, CLCNKB is linked to Bartter syndrome.