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ANGIOGENIC FACTORS

RUOLO DEI FATTORI ANGIOGENICI NELLE PATOLOGIE CISTICHE DEL FEGATO


“Cholangiopaties” are Intrahepatic biliary tree diseases including chronic and debilitating disorders of the liver that affects the pediatric population and adults. Polycystic liver disease, a group of genetic colangiopaties, are characterized by progressive dilatation of cysts of the biliary epithelium, which ultimately require a liver transplant.
he pathology of the adult dominant polycystic kidney disease (ADPKD) is caused by mutations of genes coding for ciliary proteins, the polycystins. Polycystins act functionally like mechanoreceptors, but are also involved in cell differentiation and morphogenesis of the epithelium. Our recent studies show that angiogenic growth factors, including VEGF and angiopoietin-1, are overexpressed in biliary epithelial cells (cholangiocytes) in ADPKD and may play a role in the pathogenesis of liver disease. From these observations the hypothesis of this project is that the autocrine and paracrine angiogenic signals produced by the epithelium are key triggers for the growth of cysts and liver disease progression in ADPKD.
These studies will improve our knowledge on the basic mechanisms responsible for the pathogenesis of polycystic liver disease and could allow to design strategies useful as pharmacological treatment of the disease. The results will also have a general importance to better understand the basic mechanisms that regulate angiogenesis in liver disease.