Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy H Antonicka, A Mattman, CG Carlson, DM Glerum, KC Hoffbuhr, SC Leary, ... The American Journal of Human Genetics 72 (1), 101-114, 2003 | 378 | 2003 |
Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency H Antonicka, SC Leary, GH Guercin, JN Agar, R Horvath, NG Kennaway, ... Human molecular genetics 12 (20), 2693-2702, 2003 | 306 | 2003 |
A Targetable Fluorescent Sensor Reveals That Copper-Deficient SCO1 and SCO2 Patient Cells Prioritize Mitochondrial Copper Homeostasis SC Dodani, SC Leary, PA Cobine, DR Winge, CJ Chang Journal of the American Chemical Society 133 (22), 8606-8616, 2011 | 298 | 2011 |
Human SCO1 and SCO2 have independent, cooperative functions in copper delivery to cytochrome c oxidase SC Leary, BA Kaufman, G Pellecchia, GH Guercin, A Mattman, M Jaksch, ... Human molecular genetics 13 (17), 1839-1848, 2004 | 286 | 2004 |
The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis SC Leary, PA Cobine, BA Kaufman, GH Guercin, A Mattman, J Palaty, ... Cell metabolism 5 (1), 9-20, 2007 | 267 | 2007 |
p53 improves aerobic exercise capacity and augments skeletal muscle mitochondrial DNA content JY Park, P Wang, T Matsumoto, HJ Sung, W Ma, JW Choi, SA Anderson, ... Circulation research 105 (7), 705-712, 2009 | 222 | 2009 |
Human Sco1 and Sco2 function as copper-binding proteins YC Horng, SC Leary, PA Cobine, FBJ Young, GN George, EA Shoubridge, ... Journal of Biological Chemistry 280 (40), 34113-34122, 2005 | 195 | 2005 |
Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1 SC Leary, F Sasarman, T Nishimura, EA Shoubridge Human molecular genetics 18 (12), 2230-2240, 2009 | 192 | 2009 |
Regulation of muscle mitochondrial design CD Moyes, BJ Battersby, SC Leary Journal of Experimental Biology 201 (3), 299-307, 1998 | 155 | 1998 |
Getting out what you put in: Copper in mitochondria and its impacts on human disease PA Cobine, SA Moore, SC Leary Biochimica Et Biophysica Acta (BBA)-Molecular Cell Research 1868 (1), 118867, 2021 | 153 | 2021 |
“Pulling the plug” on cellular copper: the role of mitochondria in copper export SC Leary, DR Winge, PA Cobine Biochimica et Biophysica Acta (BBA)-Molecular Cell Research 1793 (1), 146-153, 2009 | 147 | 2009 |
The mitochondrion: a central architect of copper homeostasis ZN Baker, PA Cobine, SC Leary Metallomics 9 (11), 1501-1512, 2017 | 146 | 2017 |
Interactions between bioenergetics and mitochondrial biogenesis SC Leary, BJ Battersby, RG Hansford, CD Moyes Biochimica et biophysica acta (BBA)-bioenergetics 1365 (3), 522-530, 1998 | 133 | 1998 |
The mammalian phosphate carrier SLC25A3 is a mitochondrial copper transporter required for cytochrome c oxidase biogenesis A Boulet, KE Vest, MK Maynard, MG Gammon, AC Russell, AT Mathews, ... Journal of Biological Chemistry 293 (6), 1887-1896, 2018 | 128 | 2018 |
Copper import into the mitochondrial matrix in Saccharomyces cerevisiae is mediated by Pic2, a mitochondrial carrier family protein KE Vest, SC Leary, DR Winge, PA Cobine Journal of Biological Chemistry 288 (33), 23884-23892, 2013 | 119 | 2013 |
Fiber-type differences in muscle mitochondrial profiles SC Leary, CN Lyons, AG Rosenberger, JS Ballantyne, J Stillman, ... American Journal of Physiology-Regulatory, Integrative and Comparative …, 2003 | 115 | 2003 |
Chronic treatment with azide in situ leads to an irreversible loss of cytochrome c oxidase activity via holoenzyme dissociation SC Leary, BC Hill, CN Lyons, CG Carlson, D Michaud, CS Kraft, K Ko, ... Journal of Biological Chemistry 277 (13), 11321-11328, 2002 | 107 | 2002 |
Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase M Bourens, A Boulet, SC Leary, A Barrientos Human molecular genetics 23 (11), 2901-2913, 2014 | 104 | 2014 |
Redox regulation of SCO protein function: controlling copper at a mitochondrial crossroad SC Leary Antioxidants & redox signaling 13 (9), 1403-1416, 2010 | 95 | 2010 |
Elesclomol restores mitochondrial function in genetic models of copper deficiency S Soma, AJ Latimer, H Chun, AC Vicary, SA Timbalia, A Boulet, JJ Rahn, ... Proceedings of the National Academy of Sciences 115 (32), 8161-8166, 2018 | 92 | 2018 |