PUBLICATIONS
* denotes senior author papers
2013
S.C. Leary*, Cobine, P.A., Nishimura, T., Verdijk, R.M., de Krijger, R., de Coo, R., Tarnopolsky, M.A., Winge, D.R. and Shoubridge, E.A. (2013) COX19 mediates the transduction of a mitochondrial redox signal from SCO1 that regulates ATP7A-mediated cellular copper efflux Mol. Biol. Cell mbc.E12-09-0705; First Published on January 23, 2013; doi:10.1091/mbc.E12-09-0705
2012
S.C. Leary* (2012) Blue native polyacrylamide gel electrophoresis: a powerful diagnostic tool for the detection of assembly defects in the enzyme complexes of oxidative phosphorylation. Methods Mol. Biol. 837: 195-206.
2011
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: 8606-16.
2010
S.C. Leary* (2010) Redox Regulation of SCO Protein Function: Controlling Copper at a Mitochondrial Crossroad. Antioxid Redox Signal. 13: 1403-16.
2009
Park, J.Y., Wang, P., Matsumoto, T., Sung, H.J., Ma, W., Choi, J.W., Anderson, S.A., Leary S.C., Balaban, R.S., Kang, J.G. and Hwang, P.M. (2009) Tumor suppressor p53 improves aerobic exercise capacity and augments skeletal muscle mitochondrial DNA content. Circ. Res. 105: 705-712.
Leary S.C., and Sasarman, F. (2009) Oxidative phosphorylation: synthesis of mitochondrially-encoded proteins and assembly of individual structural subunits into functional holoenzyme complexes. Methods. Mol. Biol. 554: 143-162.
Leary S.C., Sasarman. F., Nishimura, T. and Shoubridge, E.A. (2009) Human SCO2 acts as a chaperone for newly synthesized COX II synthesis and as a thiol-disulphide oxidoreductase for SCO1. Hum. Mol. Genet. 18: 2230-2240.
Leary S.C., Winge, D.R. and Cobine, P.A. (2009) “Pulling the Plug” on cellular copper: The role of mitochondria in copper export. Biochim. Biophys. Acta. 1793: 146-153.
2008
Son, M., Leary S.C., Romain, N., Pierrel, F., Winge, D.R., Haller, R.G. and Elliott, J.L. (2008) Isolated cytochrome c oxidase deficiency in G93A SOD1 mice over-expressing CCS1. J. Biol. Chem. 283: 12267-12275.
2007
Leary S.C., and Winge, D.R. (2007) The Janus face of copper: its expanding roles in biology and the pathophysiology of disease. EMBO Rep. 8: 224-227.
Leary S.C., Cobine, P.A., Kaufman, B.A., Guercin, G.-H., Mattman, A., Palaty, J., Lockitch, G., Winge, D.R., Rustin, P., Horvath, R., and Shoubridge, E.A. (2007) The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis. Cell Metab. 5: 9-20.
2006
Leary S.C., Mattman, A., Wai, T., Koehn, D.C., Clarke, S., Chan, S., Lomax, B., Eydoux, P., Vallance, H.D., and Shoubridge, E.A. (2006) A hemizygous SCO2 mutation in an early onset rapidly progressive, fatal cardiomyopathy. Mol. Genet. Metab. 89: 129-133.
Cobine, P.A., Pierrel, F., Leary S.C., Sasarman, F., Horng, Y.-C., Shoubridge, E.A. and Winge, D.R. (2006) The P174L mutation in human Sco1 severely compromises Cox17-dependent metallation but does not impair copper binding. J. Biol. Chem. 281: 12770-12776.
2003-2005
Horng, Y.-C., Leary S.C., Cobine, P.A., Young, F.B.J., George, G.N., Shoubridge, E.A., and Winge, D.R. (2005) Human Sco1 and Sco2 function as copper-binding proteins. J. Biol. Chem. 280: 34113-34122.
Leary S.C., Kaufman, B.A., Pellecchia, G., Guercin, G.H., Mattman, A., Jaksch, M. and Shoubridge, E.A. (2004) Human SCO1 and SCO2 have independent, cooperative functions in copper delivery to cytochrome c oxidase. Hum. Mol. Genet. 13: 1839-1848.
Lyons, C.N., Leary S.C., and Moyes, C.D. (2004) Bioenergetic remodeling during cellular differentiation: changes in cytochrome c oxidase regulation do not affect the metabolic phenotype. Biochem. Cell. Biochem. 82: 391-399.
Antonicka, H., Leary S.C., Guercin, G.-H., Pellecchia, G., Agar, J.N., Kennaway, N.G., Jaksch, M., and Shoubridge, E.A. (2003) Mutations in COX10 result in a defect in mitochondrial heme biosynthesis and account for multiple, early onset clinical phenotypes associated with isolated COX deficiency. Hum. Mol. Genet. 12: 2693-2702.
Leary S.C., Lyons, C.N., Rosenberger, A.G., Ballantyne, J.S., Stillman, J., and Moyes, C.D. (2003) Fiber-type differences in muscle mitochondrial profiles. Am. J. Physiol. (Regul. Integrative. Comp. Physiol.) 285: R817-R826.
Leary S.C., and Shoubridge, E.A. (2003) Mitochondrial biogenesis: which part of “NO” do we understand? Bioessays 25: 538-541.
Antonicka, H., Mattman, A., Carlson, C.G., Glerum, D.M., Hoffbuhr, K.C., Leary S.C., Kennaway, N.G., and Shoubridge, E.A. (2003) Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway causing early onset fatal hypertrophic cardiomyopathy. Am. J. Hum. Genet. 72: 101-114. J. Biol. Chem. 281: 12770-12776.
2002
Moyes, C.D., Sharma, M.L., Lyons, C.N., Leary S.C., Leon, M., Petrie, A., Lund, S., and Tufts, B. (2002) Origins and consequences of mitochondrial decline in nucleated erythrocytes. Biochim. Biophys. Acta. 1591: 11-20.
Leary S.C., Michaud, D., Lyons, C.N., Hale, T.M., Bushfield, T.L., Adams, M.A., and Moyes, C.D. (2002) Bioenergetic remodeling of heart during treatment of spontaneously hypertensive rats with enalapril. Am. J. Physiol. (Heart. Circ. Physiol.) 283: H540-H548.
Leary S.C., Hill, B.C., Lyons, C.N., Carlson, C.G., Michaud, D., Kraft, C.S., Ko, K., Glerum, D.M., and Moyes, C.D. (2002) Chronic treatment with azide in situ leads to an irreversible loss of cytochrome c oxidase activity via holoenzyme dissociation. J. Biol. Chem. 277: 11321-11328.
2000
Leary S.C., and Moyes, C.D. (2000) The effects of bioenergetic stress and redox balance on the expression of genes critical to mitochondrial function (Storey, K.B. and Storey, J.M., Eds), Cell and Molecular Responses to Stress Vol. 1 (Environmental Stressors and Gene Responses). Elsevier Science, The Netherlands, pp. 209-223.