Mitochondrial Biology&Cellular Metal Ion Homeostasis


* denotes senior author papers; _ - denotes trainee author papers; # denotes co-first authors


  • Zhu, X.#, Boulet, A.#, Buckley, K.M.#, Phillips, C.B., Gammon, M.G., Oldfather, L.E., Moore, S.A., Leary, S.C. and Cobine, P.A. (2021) Mitochondrial copper and phosphate transporter specificity was defined early in the evolution of eukaryotes. eLife Feb 16;10:e64690. doi: 10.7554/eLife.64690. Online ahead of print.
  • Quartey, M.O., Nyarko, J.N.K., Maley, J.M., Barnes, J.R., Bolanos, M.A.C., Heistad, R.M., Knudsen, K.J., Pennington, P.R., Buttigieg, J., De Carvalho, C.E., Leary, S.C., Parsons, M.P. and Mousseau, D.D. (2021) The Aβ(1-38) peptide is a negative regulator of the Aβ(1-42) peptide implicated in Alzheimer disease progression. Sci Rep. Jan 11;11(1):431. doi: 10.1038/s41598-020-80164-w..
  • Cobine, P.A.*, Moore, S.A. and Leary, S.C.* (2021) Getting out what you put in: Copper in mitochondria and its impacts on human disease. Biochim Biophys Acta Mol Cell Res. Jan;1868(1):118867. doi: 10.1016/j.bbamcr.2020.118867. Epub 2020 Oct 2.


  • Leary, S.C. and Ralle, M. (2020) Advances in visualization of copper in mammalian systems using X-ray fluorescence microscopy. Curr. Opin. Chem. Biol. 55: 19-25.


  • Soma, S., Morgada, M.N., Naik, M.T., Boulet, A., Roesler, A.A., Dziuba, N., Ghosh, A., Yu, Q., Lindahl, P.A., Ames, J.B., Leary, S.C., Vila, A.J. and Gohil, V.M. (2019) COA6 is structurally tuned to function as a thiol-disulfide oxidoreductase in copper delivery to mitochondrial cytochrome c oxidase. Cell Rep. 29: 4114-26.
  • Jin, W., Al-Dulaymi, M., Badea, I., Leary, S.C., Rehman, J. and El-Aneed, A. (2019) Cellular uptake and distribution of gemini surfactant nanoparticles used as gene delivery agents. AAPS J. 21: 98. dob: 10.1208/s12248-019-0367-1.
  • Jin, W., Badea, I., Leary, S.C. and El-Aneed, A. (2019) The determination of gemini surfactants used as gene delivery agents in cellular matrix using validated tandem mass spectrometric method. J Pharm Biomed Anal.164: 164-72.


  • Soma, S., Latimer, A.J., Chun, H., Vicary, A.C., Timbalia, S.A., Boulet, A., Rahn, J.J., Chan, S.S.L., Leary, S.C., Kim, B.E., Gitlin, J.D. and Gohil, V.M. (2018) Elesclomol restores mitochondrial function in genetic models of copper deficiency. Proc Natl Acad Sci USA 115: 8161-66.
  • Jett, K.A. and Leary, S.C.* (2018) Building the CuA site of cytochrome c oxidase: a complicated, redox-dependent process driven by a surprisingly large complient of accessory proteins. J Biol Chi 293: 4644-52.
  • Boulet, A.#, Vest, K.E.#, Maynard, M.K., Gammon, M.G., Russell, A.C., Mathews, A.T., Cole, S.E., Zhu, X., Phillips, C.B., Kwong, J.Q., Dodani, S.C., Leary, S.C., and Cobine, P.A. (2018) The mammalian phosphate carrier SLC25A3 is a mitochondrial copper transporter required for cytochrome c oxidase biogenesis. J Biol Chi 293: 1887-96.
  • Little, A.G., Lau, G., Mathers, K.E., Leary, S.C. and Moyes, C.D. (2018) Comparative biochiistry of cytochrome c oxidase in animals. Comp Biochi Physiol B (Biochi Mol Biol) 224: 170-84.


  • Baker, Z.N., Cobine, P.A. and Leary, S.C.* (2017) The mitochondrion: a central architect of copper homeostasis. Metallomics. 9: 1501-12.
  • Baker, Z.N., Jett, K., Boulet, A., Hossain, A., Cobine, P.A., Kim, B.E., El Zawily, A.M., Lee, L., Tibbits, G.F., Petris, M.J. and Leary, S.C.* (2017) The mitochondrial metallochaperone SCO1 maintains CTR1 at the plasma membrane to preserve copper homeostasis in the murine heart. Hum Mol Genet. 26: 4617-28.


  • El Zawily, A.M., Toosi, B.M., Freywald, T., Indukuri, V.V., Vizeacoumar, F.J., Leary, S.C.*, and Freywald, A.* (2016) The intrinsically kinase-inactive EPHB6 receptor predisposes cancer cells to DR5-induced apoptosis by promoting mitochondrial fragmentation. Oncotarget. 7:77865-77.
  • Xu, A., Bhanumathy, K.K., Wu, J., Ye, Z., Freywald, A., Leary, S.C., Li, R. and Xiang, J. (2016) IL-15 signaling promotes adoptive effector T-cell survival and memory formation in irradiation-induced lymphopenia. Cell Biosci. 6: 30. doi: 10.1186/s13578-016- 0098-2. eCollection 2016.


  • Pacheu-Grau, D., Bareth, B., Dudek, J., Juris, L., Vögtle, F.-N., Wissel, M., Leary, S.C., Dennerlein, S., Rehling, P. and Deckers, M. (2015) Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies. Cell Metab. 21: 823-33.
  • Hlynialuk, C.J.#, Ling, B.#, Baker, Z.N.#, Cobine, P.A., Yu, L.D., Boulet, A., Wai, T., Hossain, A., El Zawily, A.M., McFie, P.J., Stone, S.J., Diaz, F., Moraes, C.T., Viswanathan, D., Petris, M.J. and Leary, S.C.* (2015) The mitochondrial metallochaperone SCO1 is required to sustain expression of the high affinity copper transporter CTR1 and preserve copper homeostasis. Cell Reports 10: 933-43.


  • Bourens, M., Boulet, A., Leary, S.C., Barrientos, A. (2014) Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase. Hum Mol Genet. 23: 2901-13.


  • Leary, S.C., Antonicka, H., Sasarman, F., Weraarpachai, W., Cobine, P.A., Pan, M., Brown, G.K., Brown, R., Majewski, J., Ha, K.C., Rahman, S. and Shoubridge, E.A. (2013) Novel mutations in SCO1 as a cause of fatal infantile encephalopathy and lactic acidosis. Hum Mutat. 34: 1366-70.
  • Vest, K.E., Leary, S.C., Winge, D.R. and Cobine, P.A. (2013) Copper import into the mitochondrial matrix in Saccharomyces cerevisiae is mediated by Pic2, a mitochondrial carrier family protein. J Biol Chem. 33: 23884-92.
  • Leary, S.C.*, 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 24: 683-91.