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.
| Schmidt, Katharina; Ralle, Martina; Schaffer, Thomas et al. (2018) ATP7A and ATP7B copper transporters have distinct functions in the regulation of neuronal dopamine-?-hydroxylase. J Biol Chem 293:20085-20098 |
| Reed, Emily; Lutsenko, Svetlana; Bandmann, Oliver (2018) Animal models of Wilson disease. J Neurochem 146:356-373 |
| Muchenditsi, Abigael; Yang, Haojun; Hamilton, James P et al. (2017) Targeted inactivation of copper transporter Atp7b in hepatocytes causes liver steatosis and obesity in mice. Am J Physiol Gastrointest Liver Physiol 313:G39-G49 |
| Lutsenko, Svetlana (2016) Copper trafficking to the secretory pathway. Metallomics 8:840-52 |
| Hatori, Yuta; Yan, Ye; Schmidt, Katharina et al. (2016) Neuronal differentiation is associated with a redox-regulated increase of copper flow to the secretory pathway. Nat Commun 7:10640 |
| Bhattacharjee, Ashima; Yang, Haojun; Duffy, Megan et al. (2016) The Activity of Menkes Disease Protein ATP7A Is Essential for Redox Balance in Mitochondria. J Biol Chem 291:16644-58 |
| Hatori, Yuta; Lutsenko, Svetlana (2016) The Role of Copper Chaperone Atox1 in Coupling Redox Homeostasis to Intracellular Copper Distribution. Antioxidants (Basel) 5: |
| Krishnamoorthy, Lakshmi; Cotruvo Jr, Joseph A; Chan, Jefferson et al. (2016) Copper regulates cyclic-AMP-dependent lipolysis. Nat Chem Biol 12:586-92 |
| Chesi, Giancarlo; Hegde, Ramanath N; Iacobacci, Simona et al. (2016) Identification of p38 MAPK and JNK as new targets for correction of Wilson disease-causing ATP7B mutants. Hepatology 63:1842-59 |
| Wooton-Kee, Clavia Ruth; Jain, Ajay K; Wagner, Martin et al. (2015) Elevated copper impairs hepatic nuclear receptor function in Wilson's disease. J Clin Invest 125:3449-60 |
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