Thus, mutations in the PKD1 and PKD2 genes are responsible for ADPKD in humans, mutations in the PKDH1 (Polycystic kidney and hepatic disease 1) gene are responsible for ARPKD in humans, and mutations in Nek8 (NimA-related kinase 8) gene cause PKD in mice and zebrafish [32,33,34,35]. Here, PKD1 is linked to autosomal recessive polycystic kidney disease.