Mitochondria Has A Role In Cellular Copper Regulation

Their findings, published in a recent edition of the journal Cell Metabolism, identify a new role for two proteins involved with copper regulation. This study may lead to a better understanding of how to treat individuals affected by copper imbalances.

"Copper is important in maintaining healthy cells. When copper is not properly regulated in the body it can lead to diseases of the liver, kidneys, brains and eyes," says Dr. Eric Shoubridge, a professor of Human Genetics at the Montreal Neurological Institute, McGill University and lead investigator. "We know that copper is especially important in early development, playing a vital role in the proper formation of organs. Mutations in two copper-carrying proteins, SCO1 and SCO2 have been implicated in a number of neonatal diseases."

Copper is required for the activity of a number of enzymes including cytochrome c oxidase (COX) in the mitochondria -the energy suppliers of the body. "Our study is the first to characterize an unexpected cell-signaling or messenger role for the two copper-carrying proteins, SCO1 and SCO2, which are necessary for the assembly of COX," says Shoubridge.

To characterize the roles of SCO1 and SCO2, Shoubridge and colleagues looked at cells that contained mutated forms of either one or both of these molecules. The study shows that both proteins have a role in maintaining the balance of copper between different cellular compartments. "These findings add two members to a growing list of bi-functional proteins that participate in copper metabolism." adds Shoubridge. "Identifying this new role for SCO1 and SCO2 is significant in developing better therapies for several neurological diseases.