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MNI researchers find a new role for mitochondria in cellular copper regulation

Copper is an essential part of our lives. From copper pipes and wires to important copper-containing proteins in the body – copper is necessary for healthy growth and neurological development. Researchers at the Montreal Neurological Institute are studying how copper is processed in our bodies and its distinct role in early development. 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, ³ÉÈËVRÊÓƵ 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.

This study was funded by the Canadian Institutes of Health Research (CIHR) and the Muscular Dystrophy Association (MDA).

The Montreal Neurological Institute is a ³ÉÈËVRÊÓƵ research and teaching institute, dedicated to the study of the nervous system and neurological diseases. Founded in 1934 by the renowned Dr. Wilder Penfield, the MNI is one of the world's largest institutes of its kind. MNI researchers are world leaders in cellular and molecular neuroscience, brain imaging, cognitive neuroscience and the study and treatment of epilepsy, multiple sclerosis and neuromuscular disorders. The MNI, with its clinical partner, the Montreal Neurological Hospital (MNH), part of the ³ÉÈËVRÊÓƵ Health Centre, continues to integrate research, patient care and training, and is recognized as one of the premier neuroscience centres in the world.