Integrative Analysis of Wilson's Disease

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PROJECT SUMMARY The LSM800 instrument will be heavily used for all specific aims of the parental grant. The studies that will be performed with the microscope include analysis of trafficking of ATP7A and ATP7B between the cell compartments, regulation of the trafficking by norepinephrine and other signaling molecules, intracellular measurements of coper transport activity, studies of the redox environment in live cells, and evaluation of tissue morphology with a quantitative analysis of cell densities. Studies in live cells would allow more sophisticated kinetic measurements as well as evaluation of spatial aspects of DBH secretion, which has not been done previously. The ability to re-use several components of the Pascal microscope, which is no longer supported by the manufacturer, resulted in a significant discount in the price of the microscope, despite addition of the live-cell imaging option, which is highly beneficial for the proposed research.