With respect to this idea, we and others have previously reported that inactivating MAGE-D2, a protein that protects NKCC2 and NCC against ER associated degradation, leads to BS type 5, which is the most severe form of antenatal Bartter syndrome described so far, as judged by both onset and severity of polyhydramnios and the high rate of mortality observed in patient carriers of MAGE-D2 mutations [28,47,48]. Here, MAGED2 is linked to Bartter syndrome.