Consequently, deletion of AC6 but not AC3 specifically in PCs results in a urine concentrating defect in mice that mimics global knockout of AC6 and nephrogenic diabetes insipidus (Rieg et al., 2010; Roos et al., 2012; Kittikulsuth et al., 2014). The gene discussed is ADCY3; the disease is nephrogenic diabetes insipidus.