Autosomal dominant polycystic kidney disease (ADPKD) is an inherited systemic disorder caused by mutations in PKD1 or PKD2 that predominately affects the kidneys and is characterized by the formation of cysts in the kidneys and other organs. Xia Zhou and colleagues used a mouse model of ADPKD to explore the role of the protein sirtuin 1 in cyst formation. In the accompanying image, slices of kidney from (left to right) mice lacking sirtuin 1, ADPKD mice, mice lacking 1 copy of sirtuin 1 gene, and mice lacking both copies of the sirtuin 1 gene, were fixed and stained with hematoxylin to visualize the ADPKD-associated cysts. These data demonstrate that loss of both copies of sirtuin 1 reduces cyst formation in ADPKD. Interestingly, the activity of sirtuin 1 can be inhibited by treatment with vitamin B3, suggesting that this vitamin may also be beneficial in ADPKD.
Autosomal-dominant polycystic kidney disease (ADPKD) is caused by mutations in either
Xia Zhou, Lucy X. Fan, William E. Sweeney Jr., John M. Denu, Ellis D. Avner, Xiaogang Li