STAT signaling in polycystic kidney disease

The most common form of polycystic kidney disease (PKD) in humans is caused by mutations in the PKD1 gene coding for polycystin1 (PC1). Among the many identified or proposed functions of PC1 is its ability to regulate the activity of transcription factors of the STAT family. Most STAT proteins that have been investigated were found to be aberrantly activated in kidneys in PKD, and some have been shown to be drivers of disease progression. In this review, we focus on the role of signal transducer and activator of transcription (STAT) signaling pathways in various renal cell types in healthy kidneys as compared to polycystic kidneys, on the mechanisms of STAT regulation by PC1 and other factors, and on the possibility to target STAT signaling for PKD therapy. •Most STAT signaling pathways are activated in kidneys in PKD.•STAT signaling is regulated by polycystin-1, the protein affected by mutations in ADPKD.•STAT signaling pathways can be activated in different renal cell types leading to different effects.•Inhibitors of STAT signaling may be promising for PKD therapy.