In contrast to ADPKD, where genes encode proteins that localize to the primary cilium, the gene products implicated in ADPLD (PRKCSH, SEC63, SEC61A, SEC61B, GANAB, ALG8, and ALG9) are all involved in the co-translational translocation and maturation of glycoproteins in the endoplasmic reticulum [15,16,24]. Here, PRKCSH is linked to autosomal dominant polycystic liver disease.