INTERACTIONS BETWEEN THE COG COMPLEX AND ATP7A IN NEURODEGENERATIVE DISEASE
Menkes disease is an X-linked neurodegenerative disease related to the improper distribution of copper. This disease can result from mutations in the ATP7A copper transporter gene. In the Golgi apparatus, ATP7A imports copper used in the assembly of cuproenzymes and if copper levels are too high, ATP7A localizes to the cell membrane and functions to pump copper out of the cell. Because of this, when ATP7A is not functioning properly, copper cannot be distributed to the brain and other tissues in the body. Other proteins have been found to interact with ATP7A for the regulation of copper with one being the Conserved Oligomeric Golgi (COG) Complex. We are looking at how the COG complex interacts with ATP7A's ability to transport copper. For this study, we focus on COG1, COG5, and COG8, three members of the COG complex. We inhibited each member of the COG complex in dopaminergic neurons and assayed for copper sensitivity. We found that inhibition of the COG complex leads to decreased sensitivity to copper exposure. In addition, we found that the COG complex interacts with ATP7A to regulate copper sensitivity.
Margewich, Lindsey, "INTERACTIONS BETWEEN THE COG COMPLEX AND ATP7A IN NEURODEGENERATIVE DISEASE" (2019). University Research Symposium. 201.