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Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1.
Leary SC, Sasarman F, Nishimura T, Shoubridge EA. Leary SC, et al. Hum Mol Genet. 2009 Jun 15;18(12):2230-40. doi: 10.1093/hmg/ddp158. Epub 2009 Mar 31. Hum Mol Genet. 2009. PMID: 19336478
Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies.
Pacheu-Grau D, Bareth B, Dudek J, Juris L, Vögtle FN, Wissel M, Leary SC, Dennerlein S, Rehling P, Deckers M. Pacheu-Grau D, et al. Among authors: leary sc. Cell Metab. 2015 Jun 2;21(6):823-33. doi: 10.1016/j.cmet.2015.04.012. Epub 2015 May 7. Cell Metab. 2015. PMID: 25959673
Redox regulation of SCO protein function: controlling copper at a mitochondrial crossroad.
Leary SC. Leary SC. Antioxid Redox Signal. 2010 Nov 1;13(9):1403-16. doi: 10.1089/ars.2010.3116. Antioxid Redox Signal. 2010. PMID: 20136502 Review.
A targetable fluorescent sensor reveals that copper-deficient SCO1 and SCO2 patient cells prioritize mitochondrial copper homeostasis.
Dodani SC, Leary SC, Cobine PA, Winge DR, Chang CJ. Dodani SC, et al. Among authors: leary sc. J Am Chem Soc. 2011 Jun 8;133(22):8606-16. doi: 10.1021/ja2004158. Epub 2011 May 12. J Am Chem Soc. 2011. PMID: 21563821 Free PMC article.
The mammalian phosphate carrier SLC25A3 is a mitochondrial copper transporter required for cytochrome c oxidase biogenesis.
Boulet A, Vest KE, Maynard MK, Gammon MG, Russell AC, Mathews AT, Cole SE, Zhu X, Phillips CB, Kwong JQ, Dodani SC, Leary SC, Cobine PA. Boulet A, et al. Among authors: leary sc. J Biol Chem. 2018 Feb 9;293(6):1887-1896. doi: 10.1074/jbc.RA117.000265. Epub 2017 Dec 13. J Biol Chem. 2018. PMID: 29237729 Free PMC article.
Human Sco1 and Sco2 function as copper-binding proteins.
Horng YC, Leary SC, Cobine PA, Young FB, George GN, Shoubridge EA, Winge DR. Horng YC, et al. Among authors: leary sc. J Biol Chem. 2005 Oct 7;280(40):34113-22. doi: 10.1074/jbc.M506801200. Epub 2005 Aug 9. J Biol Chem. 2005. PMID: 16091356
The P174L mutation in human Sco1 severely compromises Cox17-dependent metallation but does not impair copper binding.
Cobine PA, Pierrel F, Leary SC, Sasarman F, Horng YC, Shoubridge EA, Winge DR. Cobine PA, et al. Among authors: leary sc. J Biol Chem. 2006 May 5;281(18):12270-6. doi: 10.1074/jbc.M600496200. Epub 2006 Mar 6. J Biol Chem. 2006. PMID: 16520371
The mitochondrial metallochaperone SCO1 maintains CTR1 at the plasma membrane to preserve copper homeostasis in the murine heart.
Baker ZN, Jett K, Boulet A, Hossain A, Cobine PA, Kim BE, El Zawily AM, Lee L, Tibbits GF, Petris MJ, Leary SC. Baker ZN, et al. Among authors: leary sc. Hum Mol Genet. 2017 Dec 1;26(23):4617-4628. doi: 10.1093/hmg/ddx344. Hum Mol Genet. 2017. PMID: 28973536 Free PMC article.
Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase.
Bourens M, Boulet A, Leary SC, Barrientos A. Bourens M, et al. Among authors: leary sc. Hum Mol Genet. 2014 Jun 1;23(11):2901-13. doi: 10.1093/hmg/ddu003. Epub 2014 Jan 8. Hum Mol Genet. 2014. PMID: 24403053 Free PMC article.
Novel mutations in SCO1 as a cause of fatal infantile encephalopathy and lactic acidosis.
Leary SC, Antonicka H, Sasarman F, Weraarpachai W, Cobine PA, Pan M, Brown GK, Brown R, Majewski J, Ha KC, Rahman S, Shoubridge EA. Leary SC, et al. Hum Mutat. 2013 Oct;34(10):1366-70. doi: 10.1002/humu.22385. Epub 2013 Aug 12. Hum Mutat. 2013. PMID: 23878101
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