Quinomycin A reduces cyst progression in polycystic kidney disease

Polycystic kidney disease (PKD) is a genetic disorder characterized by aberrant renal epithelial cell proliferation and formation progressive growth of numerous fluid-filled cysts within the kidneys. Previously, we showed that there elevated Notch signaling compared to normal cells contributes cystic cells. Quinomycin A, bis-intercalator peptide, has previously been shown target pathway inhibit tumor in cancer. Here, show A decreased cyst human ADPKD cultured 3D collagen gel. Treatment with reduced weight body ratio area fibrosis Pkd1RC/RC; Pkd2+/− mice, an orthologous PKD mouse model. This was accompanied expression proteins, RBPjk HeyL kidneys mice. treatments also normalized cilia length derived from collecting ducts. first study demonstrate effectively inhibits progression suggests potential therapeutic value for patients.