Diseases of the liver affect thousands of people annually. The cholangiopathies are a group of diseases involving the cells, called cholangiocytes, that line the bile ducts of the liver. The only cure for these kinds of liver diseases is a liver transplant, which is not always a viable option.
The goal of Dr. LaRusso's Cholangiopathies Laboratory is to increase the understanding of the structure, function and dysfunction of these cells by studying the process of bile formation and the possible role of the cholangiocyte primary cilia in this process. The Cholangiopathies lab uses two animal models of autosomal recessive kidney and liver disease and one model of autosomal dominant kidney and liver disease for the study of abnormalities of cholangiocyte cilia that lead to the formation of liver cysts. The Cholangiopathies lab's experimental models and methods provide the basis for developing new therapies and drugs to treat these devastating liver diseases.