Integrative Analysis of Wilson's Disease

项目摘要 LSM 800工具将大量用于父母补助金的所有具体目标。研究 包括分析ATP 7A和ATP 7B的运输 在细胞间室之间，通过去甲肾上腺素和其他信号调节运输 分子，铜转运活性的细胞内测量，氧化还原的研究 活细胞中的环境，并通过定量分析细胞的形态学来评价组织形态学。 密度活细胞研究将允许更复杂的动力学测量， DBH分泌的空间方面的评价，这是以前没有做过的。的能力 重新使用Pascal显微镜的几个组件，该显微镜不再受 制造商，导致显微镜的价格显着折扣，尽管增加了 活细胞成像选项，这对拟议的研究非常有益。

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.