Gene Symbol: SCO1
Description: Cu-binding protein SCO1
Alias: PET161, Cu-binding protein SCO1
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Rigby K, Cobine P, Khalimonchuk O, Winge D. Mapping the functional interaction of Sco1 and Cox2 in cytochrome oxidase biogenesis. J Biol Chem. 2008;283:15015-22 pubmed publisher
    b>Sco1 is implicated in the copper metallation of the Cu(A) site in Cox2 of cytochrome oxidase. The structure of Sco1 in the metallated and apo-conformers revealed structural dynamics primarily in an exposed region designated loop 8...
  2. Abajian C, Rosenzweig A. Crystal structure of yeast Sco1. J Biol Inorg Chem. 2006;11:459-66 pubmed
    ..The crystal structures of Saccharomyces cerevisiae apo Sco1 (apo-ySco1) and Sco1 in the presence of copper ions (Cu-ySco1) were determined to 1.8- and 2...
  3. Buchwald P, Krummeck G, Rödel G. Immunological identification of yeast SCO1 protein as a component of the inner mitochondrial membrane. Mol Gen Genet. 1991;229:413-20 pubmed
    The SCO1 gene of Saccharomyces cerevisiae encodes a 30 kDa protein which is specifically required for a post-translational step in the accumulation of subunits 1 and 2 of cytochrome c oxidase (COXI and COX-II)...
  4. Glerum D, Shtanko A, Tzagoloff A. SCO1 and SCO2 act as high copy suppressors of a mitochondrial copper recruitment defect in Saccharomyces cerevisiae. J Biol Chem. 1996;271:20531-5 pubmed
    ..In the present study we show that the respiratory defect of C129/U1 is rescuable by two multicopy suppressors, SCO1 and SCO2...
  5. Lode A, Kuschel M, Paret C, Rödel G. Mitochondrial copper metabolism in yeast: interaction between Sco1p and Cox2p. FEBS Lett. 2000;485:19-24 pubmed
    ..These amino acids, which are conserved among the members of the Sco1p family, have been suggested to act in the reduction of the cysteines in the copper binding center of Cox2p and are discussed as ligands for copper. ..
  6. Williams J, Sue C, Banting G, Yang H, Glerum D, Hendrickson W, et al. Crystal structure of human SCO1: implications for redox signaling by a mitochondrial cytochrome c oxidase "assembly" protein. J Biol Chem. 2005;280:15202-11 pubmed
    Human SCO1 and SCO2 are copper-binding proteins involved in the assembly of mitochondrial cytochrome c oxidase (COX). We have determined the crystal structure of the conserved, intermembrane space core portion of apo-hSCO1 to 2.8 A...
  7. Gamberi T, Magherini F, Borro M, Gentile G, Cavalieri D, Marchi E, et al. Novel insights into phenotype and mitochondrial proteome of yeast mutants lacking proteins Sco1p or Sco2p. Mitochondrion. 2009;9:103-14 pubmed publisher
    ..Our results indicated a higher fermentative capacity of the sco1-Delta mutant in comparison to the control and the sco2-Delta mutant strains...
  8. Khalimonchuk O, Bird A, Winge D. Evidence for a pro-oxidant intermediate in the assembly of cytochrome oxidase. J Biol Chem. 2007;282:17442-9 pubmed
    The hydrogen peroxide sensitivity of cells lacking two proteins, Sco1 and Cox11, important in the assembly of cytochrome c oxidase (CcO), is shown to arise from the transient accumulation of a pro-oxidant heme A-Cox1 stalled intermediate...
  9. Lode A, Paret C, Rödel G. Molecular characterization of Saccharomyces cerevisiae Sco2p reveals a high degree of redundancy with Sco1p. Yeast. 2002;19:909-22 pubmed
    The Saccharomyces cerevisiae gene SCO1 has been shown to play an essential role in the transfer of copper to the Cu(A)-centre of the mitochondrial cytochrome c oxidase subunit Cox2p...

More Information


  1. Khalimonchuk O, Ostermann K, Rödel G. Evidence for the association of yeast mitochondrial ribosomes with Cox11p, a protein required for the Cu(B) site formation of cytochrome c oxidase. Curr Genet. 2005;47:223-33 pubmed
    ..We propose a model in which the Cu(B) site is co-translationally formed by a transient interaction between Cox11p and the nascent Cox1p in the intermembrane space. ..
  2. Ghosh A, Pratt A, Soma S, Theriault S, Griffin A, Trivedi P, et al. Mitochondrial disease genes COA6, COX6B and SCO2 have overlapping roles in COX2 biogenesis. Hum Mol Genet. 2016;25:660-71 pubmed publisher
    ..Taken together, these results place COA6 in the copper delivery pathway to CcO and, surprisingly, link it to a previously unidentified function of CcO subunit Cox12 in Cox2 biogenesis. ..
  3. Glerum D, Shtanko A, Tzagoloff A. Characterization of COX17, a yeast gene involved in copper metabolism and assembly of cytochrome oxidase. J Biol Chem. 1996;271:14504-9 pubmed
    ..Evidence is presented indicating that Cox17p is not involved in general copper metabolism in yeast but rather has a more specific function in the delivery of copper to mitochondria. ..
  4. Chinenov Y. Cytochrome c oxidase assembly factors with a thioredoxin fold are conserved among prokaryotes and eukaryotes. J Mol Med (Berl). 2000;78:239-42 pubmed
    ..b>Sco1/2 proteins have been isolated as high-copy number suppressors of a deletion of copper chaperone Cox 17, implicating ..
  5. Chojnacka M, Gornicka A, Oeljeklaus S, Warscheid B, Chacinska A. Cox17 Protein Is an Auxiliary Factor Involved in the Control of the Mitochondrial Contact Site and Cristae Organizing System. J Biol Chem. 2015;290:15304-12 pubmed publisher
    ..Cox17 interacts with Mic60, thereby modulating MICOS complex integrity. This interaction does not involve Sco1, a partner of Cox17 in transferring copper ions to cytochrome c oxidase...
  6. Veniamin S, Sawatzky L, Banting G, Glerum D. Characterization of the peroxide sensitivity of COX-deficient yeast strains reveals unexpected relationships between COX assembly proteins. Free Radic Biol Med. 2011;51:1589-600 pubmed publisher
    ..Using a quantitative assay, we have identified subtle differences in the peroxide-sensitive phenotypes between sco1 and cox11 mutant strains...
  7. Pacheu Grau D, Bareth B, Dudek J, Juris L, Vögtle F, Wissel M, et al. Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies. Cell Metab. 2015;21:823-33 pubmed publisher
    ..Our analyses define COA6 as a constituent of the mitochondrial copper relay system, linking defects in COX2 metallation to cardiac cytochrome c oxidase deficiency. ..
  8. Schulze M, Rödel G. SCO1, a yeast nuclear gene essential for accumulation of mitochondrial cytochrome c oxidase subunit II. Mol Gen Genet. 1988;211:492-8 pubmed
    We have identified and isolated a novel yeast nuclear gene (SCO1) which is essential for accumulation of the mitochondrially synthesized subunit II of cytochrome c oxidase (CoxII)...
  9. Nittis T, George G, Winge D. Yeast Sco1, a protein essential for cytochrome c oxidase function is a Cu(I)-binding protein. J Biol Chem. 2001;276:42520-6 pubmed
    b>Sco1 is a conserved essential protein, which has been implicated in the delivery of copper to cytochrome c oxidase, the last enzyme of the electron transport chain...
  10. Steinebrunner I, Landschreiber M, Krause Buchholz U, Teichmann J, Rödel G. HCC1, the Arabidopsis homologue of the yeast mitochondrial copper chaperone SCO1, is essential for embryonic development. J Exp Bot. 2011;62:319-30 pubmed publisher
    The Arabidopsis HCC1 gene is a homologue of the copper chaperone SCO1 from the yeast Saccharomyces cerevisiae...