PKD1 and autosomal dominant polycystic kidney disease: In addition, Cai and colleagues described the effect of several mutations in Pkd1 and Pkd2 in the importance of PCs trafficking to cilia using in vitro and in vivo models, concluding that altered trafficking and dysfunctional maturation of PC complex underlie PKD pathology (71) These facts suggest a central role for PC1 in the cystogenesis process and in regulating the severity of ADPKD, ARPKD, and ADPLD (72).