In the case of a germline PRKCSH mutation in ADPLD, the second hit could involve a somatic PRKCSH or SEC63 mutation on the other allele, or somatic hits on genes implicated in Autosomal Dominant Polycystic Kidney Disease (ADPKD), such as PKD1 and PKD216. The gene discussed is PRKCSH; the disease is autosomal dominant polycystic liver disease.