CLCN5 and nephrocalcinosis: However, targeted disruption of Clcn5 in the Jentsch model did not lead to hypercalciuria, one of the hallmark of Dent disease that is present in approximately 80% of patients with DD1, while a similar disruption in the Guggino model led to hypercalciuria and nephrocalcinosis.5 How ClC-5 loss-of-function in PT could cause elevated urinary calcium is still not completely understood.