3D Microperfusion Model of ADPKD

Abstract : Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a hereditary disorder associated with a mutation of either PKD1 or PKD2. The pathophysiological mechanisms of ADPKD which ultimately result in the formation of bilateral focal cysts are not well understood. Specifically, the early disease pathways associated with the transition for a tubule to cystic phenotype are not well understood and consequently no targeted treatments for ADPKD exist. The purpose of this project is to develop and characterize a physiologically relevant platform that combines a 3D culture environment, which allows for changes in morphological phenotype, and fluid induced shear stresses that mimic the mechanical forces found in the kidney. The focus of the research was specifically targeted around developing the perfusion system and characterizing the cell types necessary for developing an in vitro perfusion model of ADPKD. The ultimate goal of the research is to characterize normal and disease phenotypes within the model system.