Ph.D., 2001, Queen's University, Department of Biology B.Sc.., 1995, University of Guelph, Department of Zoology Mitochondria are cellular organelles that have long been recognized for their essential role in producing ATP, the chemical form of energy required to fuel a diverse range of biochemical reactions within the cell. In the last decade or so, however, additional roles for mitochondria in a myriad of other homeostatic pathways within the cell have been identified (e.g. apoptosis, non-shivering thermogenesis, iron- sulphur cluster biogenesis, cellular copper homeostasis). We are interested in identifying and characterizing the molecular genetic mechanisms that allow mitochondria to make so many varied, and fundamental, contributions to cell biology.

Bourens, M., A. Boulet, S.C. Leary and A. Barrientos. 2014. Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase. Hum. Mol. Genet. 23: 2901-2913. Vest, K.E., S.C. Leary, D.R. Winge and P.A. Cobine. 2013. Pic2 mediates import of copper into the mitochondrial matrix in Saccharomyces cerevisiae. J. Biol. Chem. 288: 23884-23892. Leary, S.C.,* H. Antonicka,* F. Sasarman, W. Weraarpachai, P.A. Cobine, M. Pan, G.K. Brown, R. Brown, J. Majewski, J. Swartzentruber, S. Rahman, and E.A. Shoubridge. 2013. Novel mutations in SCO1 as a cause of fatal infantile encephalopathy and lactic acidosis. Hum. Mutat. 34: 1366-1370. Leary, S.C.,† P.A. Cobine, T. Nishimura, R.M. Verdijk, R. de Krijger, R. de Coo, M.A. Tarnopolsky, D.R. Winge and E.A. Shoubridge. 2013. COX19 mediates the transduction of a mitochondrial redox signal from SCO1 that regulates ATP7A-mediated cellular copper efflux. Mol. Biol. Cell. 24: 683-691. Dodani, S.C., Leary, S.C., Cobine, P.A., Winge, D.R. and Chang, C.J. 2011. A targetable fluorescent sensor reveals that copper-deficient SCO1 and SCO2 patient cells prioritize mitochondrial copper homeostasis. J. Am. Chem. Soc. 133: 8606-8616.