Beyond PKD1 and PKD2 (which present with high levels of allelic heterogeneity [51]), a series of mutated genes has been described in ADPKD, such as neutral α-glucosidase AB (GANAB) [52], DNAJB11 [53], SEC63, and PRKCSH [54] (Figure 2). Here, PKD2 is linked to autosomal dominant polycystic kidney disease.