Particularly, we found that cystic cell-derived EVs/exosomes and urinary exosomes derived from ADPKD patients not only affected recipient cell function but also promoted cyst growth in Pkd1 mutant mice and in 3D cultures by the downregulation of Pkd1 gene expression and upregulation of miRNAs, including miR-200s and miR-21, leading to the activation of PKD-associated signaling pathways. Here, PKD1 is linked to cyst.