The pathogenesis and treatment of autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disease which can occur at any age without obvious racial or gender preferences. Generally, the PKD1 and PKD2 genes are considered as the pathogenic genes for ADPKD. However, there are still some ADPKD patients in whom no pathogenic gene is detected. The PKD1 and PKD2 genes encode polycystic protein 1 (polycystin 1, PC1) and polycystic protein 2 (polycystin 2, PC2), respectively. PC1 and PC2 combine to form PC1/PC2 complex that is involved in regulating several common cell signaling pathways. At present, the treatment of ADPKD is mainly to delay the occurrence and progression of this disease. In the clinical, surgical treatment are common method and are effective. We present a review emphasizing the pathogenesis and current treatment of this condition. Key words: Autosomal dominant polycystic kidney disease; Pathogenic gene; Pathogenesis; Drug therapy