IG Researcher profile: Scot C. Leary

Researcher profiles

Project title

Functional genetic investigation of the mitochondrial regulation of copper homeostasis: This project deals with the two-sided nature of copper, by identifying and understanding the mechanisms that ensure the appropriate handling of this essential, but potentially toxic, nutrient.

Who

Dr. Scot Leary is an Assistant Professor, in the Department of Biochemistry within the College of Medicine at the University of Saskatchewan.

Issue

It is estimated that a metal ion is needed as a structural or catalytic co-factor for the proper function of roughly one third of our proteome. Copper is one of the most commonly used metals in biology, and we get this essential trace element from our diet. Environmental insults or genetic lesions that prevent us from getting, distributing or handling copper collectively result in severe forms of early and late-onset disease. Copper is also a critical factor in many host-pathogen interactions, cell cycle progression and tumorigenesis. Nevertheless, we know surprisingly little about the homeostatic mechanisms that allow for the safe acquirement and management of copper within the cell and entire body.

Approach

The mitochondrion is a small compartment within the cell that fulfills many functions crucial to cellular homeostasis. Dr. Leary’s lab investigates how the organelle is able to make fundamental contributions to the regulation of copper handling at the cellular and the systemic levels of organization, by studying immortalized patient cell lines derived from pediatric cases and transgenic mouse models of the human disease.

Impact

This research strives to identify the mechanisms by which copper handling pathways, contained within mitochondria, communicate with those localized elsewhere in the cell to regulate copper homeostasis. This work will deepen our appreciation of how mitochondria contribute to the safe handling and appropriate distribution of copper throughout the cell and the entire body. This in turn, may lead to the development of innovative therapies for the effective treatment of human diseases caused by defects in the associated copper handling pathways.

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