Rare variants in genes such as PKRSCH, SEC61B and SEC63 have been attributed to ADPLD, but should also be considered when making a molecular genetic diagnosis in patients with kidney cysts due to the interlinking nature of the pathogenesis mechanism of kidney and liver cysts, resulting from perturbed post-translational polycystin-1 modification, reducing functional polycystin-1 levels [12]. This evidence concerns the gene PKD1 and autosomal dominant polycystic liver disease.