Rare pathogenic variants in other genes were also examined in 11 patients with variants of uncertain significance in PKD1, PKD2, or GANAB in a similar way, and while the functional roles in ADPKD were undetermined for most of these variants, the roles of CPLANE1 in patient 18 and AHI1 in patient 47, both of which were cilia-associated genes, might be involved in the development of ADPKD to some degree. This evidence concerns the gene CPLANE1 and autosomal dominant polycystic kidney disease.