Researchers from Yale University School of Medicine have discovered a new mechanism that affects both the severity and progression of PKD. Previously, cyst growth has been linked to mutations in the PKD1 and PKD2 genes and their associated proteins, polycystin-1 and -2. Scientists have found that defective cilia in renal tube cells also play a part in the disease. The Yale researchers have shown yet another way that polycystins and cilia affect the development of PKD. In their experiments, PKD cysts grew in mice with intact cilia and inactive polycystins. When the scientists inactivated or disrupted the cilia as well, the cysts stopped growing, suggesting a promising target for future treatments of PKD.
Sources:
Surprising Mechanism Discovered in Polycystic Kidney Disease, Yale News, Helen Dodson, July 26, 2013
Abstract:
Loss of Cilia Suppresses Cyst Growth in Genetic Models of Autosomal Dominant Polycystic Kidney Disease, Nature Genetics, July 28, 2013