Gene Symbol: ERV1
Description: flavin-linked sulfhydryl oxidase
Alias: flavin-linked sulfhydryl oxidase
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Ang S, Lu H. Deciphering structural and functional roles of individual disulfide bonds of the mitochondrial sulfhydryl oxidase Erv1p. J Biol Chem. 2009;284:28754-61 pubmed publisher
    ..We show that both CXXC disulfides are required for Erv1 oxidase activity. The active site disulfide is well protected thus requires the shuttle disulfide for its function...
  2. Kawano S, Yamano K, Naoé M, Momose T, Terao K, Nishikawa S, et al. Structural basis of yeast Tim40/Mia40 as an oxidative translocator in the mitochondrial intermembrane space. Proc Natl Acad Sci U S A. 2009;106:14403-7 pubmed publisher
    ..These results are consistent with the growth phenotypes of Mia40 mutant cells containing Ser replacement of the conserved cysteine residues. ..
  3. Grumbt B, Stroobant V, Terziyska N, Israel L, Hell K. Functional characterization of Mia40p, the central component of the disulfide relay system of the mitochondrial intermembrane space. J Biol Chem. 2007;282:37461-70 pubmed
    ..In summary, we reconstituted a disulfide relay system consisting of Mia40C and Erv1p. ..
  4. Chacinska A, Guiard B, Müller J, Schulze Specking A, Gabriel K, Kutik S, et al. Mitochondrial biogenesis, switching the sorting pathway of the intermembrane space receptor Mia40. J Biol Chem. 2008;283:29723-9 pubmed publisher
  5. Lange H, Lisowsky T, Gerber J, Muhlenhoff U, Kispal G, Lill R. An essential function of the mitochondrial sulfhydryl oxidase Erv1p/ALR in the maturation of cytosolic Fe/S proteins. EMBO Rep. 2001;2:715-20 pubmed
    ..It is likely that Erv1p/ALR operates downstream of the mitochondrial ABC transporter Atm1p/ABC7/Sta1, which also executes a specific task in this essential biochemical process. ..
  6. Bourens M, Dabir D, Tienson H, Sorokina I, Koehler C, Barrientos A. Role of twin Cys-Xaa9-Cys motif cysteines in mitochondrial import of the cytochrome C oxidase biogenesis factor Cmc1. J Biol Chem. 2012;287:31258-69 pubmed publisher
    ..can be oxidized by the import receptor Mia40 alone when in excess or at a lower rate by only the sulfhydryl oxidase Erv1. However, physiological and efficient Cmc1 oxidation requires Erv1 and Mia40...
  7. Tienson H, Dabir D, Neal S, Loo R, Hasson S, Boontheung P, et al. Reconstitution of the mia40-erv1 oxidative folding pathway for the small tim proteins. Mol Biol Cell. 2009;20:3481-90 pubmed publisher
    Mia40 and Erv1 execute a disulfide relay to import the small Tim proteins into the mitochondrial intermembrane space...
  8. Terziyska N, Grumbt B, Kozany C, Hell K. Structural and functional roles of the conserved cysteine residues of the redox-regulated import receptor Mia40 in the intermembrane space of mitochondria. J Biol Chem. 2009;284:1353-63 pubmed publisher
    ..a disulfide relay system whose key components are the redox-regulated import receptor Mia40 and the thiol oxidase Erv1. Mia40 contains six cysteine residues in a CPC-CX9C-CX9C arrangement in a highly conserved domain...
  9. Stojanovski D, Milenkovic D, Müller J, Gabriel K, Schulze Specking A, Baker M, et al. Mitochondrial protein import: precursor oxidation in a ternary complex with disulfide carrier and sulfhydryl oxidase. J Cell Biol. 2008;183:195-202 pubmed publisher
    ..represents a transient and intermediate step in the oxidation of intermembrane space precursors, where the oxidase Erv1 promotes disulfide transfer to the precursor while both oxidase and precursor are associated with the disulfide ..

More Information


  1. Müller J, Milenkovic D, Guiard B, Pfanner N, Chacinska A. Precursor oxidation by Mia40 and Erv1 promotes vectorial transport of proteins into the mitochondrial intermembrane space. Mol Biol Cell. 2008;19:226-36 pubmed
    ..Import of the precursors requires the essential intermembrane space proteins Mia40 and Erv1 that were proposed to form a relay for disulfide formation in the precursor proteins...
  2. Rissler M, Wiedemann N, Pfannschmidt S, Gabriel K, Guiard B, Pfanner N, et al. The essential mitochondrial protein Erv1 cooperates with Mia40 in biogenesis of intermembrane space proteins. J Mol Biol. 2005;353:485-92 pubmed
    ..Here, we show that Erv1, a further essential IMS protein that has been reported to function as a sulfhydryl oxidase and participate in ..
  3. Mesecke N, Terziyska N, Kozany C, Baumann F, Neupert W, Hell K, et al. A disulfide relay system in the intermembrane space of mitochondria that mediates protein import. Cell. 2005;121:1059-69 pubmed
    ..Mia40 contains cysteine residues, which are oxidized by the sulfhydryl oxidase Erv1. Depletion of Erv1 or conditions reducing Mia40 prevent protein import...
  4. Guo P, Ma J, Jiang Y, Wang S, Bao Z, Yu X, et al. Structure of yeast sulfhydryl oxidase erv1 reveals electron transfer of the disulfide relay system in the mitochondrial intermembrane space. J Biol Chem. 2012;287:34961-9 pubmed publisher
    ..Reduced Mia40 is reoxidized/regenerated by the FAD-linked sulfhydryl oxidase Erv1 (EC
  5. Böttinger L, Gornicka A, Czerwik T, Bragoszewski P, Loniewska Lwowska A, Schulze Specking A, et al. In vivo evidence for cooperation of Mia40 and Erv1 in the oxidation of mitochondrial proteins. Mol Biol Cell. 2012;23:3957-69 pubmed publisher formation pathway is based on a relay of reactions involving disulfide transfer from the sulfhydryl oxidase Erv1 to Mia40 and from Mia40 to substrate proteins...
  6. Lee J, Hofhaus G, Lisowsky T. Erv1p from Saccharomyces cerevisiae is a FAD-linked sulfhydryl oxidase. FEBS Lett. 2000;477:62-6 pubmed
    The yeast ERV1 gene encodes a small polypeptide of 189 amino acids that is essential for mitochondrial function and for the viability of the cell...
  7. Lionaki E, Aivaliotis M, Pozidis C, Tokatlidis K. The N-terminal shuttle domain of Erv1 determines the affinity for Mia40 and mediates electron transfer to the catalytic Erv1 core in yeast mitochondria. Antioxid Redox Signal. 2010;13:1327-39 pubmed publisher
    b>Erv1 and Mia40 constitute the two important components of the disulfide relay system that mediates oxidative protein folding in the mitochondrial intermembrane space...
  8. Bien M, Longen S, Wagener N, Chwalla I, Herrmann J, Riemer J. Mitochondrial disulfide bond formation is driven by intersubunit electron transfer in Erv1 and proofread by glutathione. Mol Cell. 2010;37:516-28 pubmed publisher
    ..Major players in this pathway are the oxidoreductase Mia40 that oxidizes substrates and the sulfhydryl oxidase Erv1 that reoxidizes Mia40...
  9. Dabir D, Leverich E, Kim S, Tsai F, Hirasawa M, Knaff D, et al. A role for cytochrome c and cytochrome c peroxidase in electron shuttling from Erv1. EMBO J. 2007;26:4801-11 pubmed
    b>Erv1 is a flavin-dependent sulfhydryl oxidase in the mitochondrial intermembrane space (IMS) that functions in the import of cysteine-rich proteins...
  10. 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
    ..However, the Cox17-MICOS interaction is regulated by copper ions. We propose that Cox17 is a newly identified factor involved in maintaining the architecture of the MICOS complex. ..
  11. Wang Q, Ang S, Ceh Pavia E, Pang J, Lu H. Role of tryptophan residues of Erv1: Trp95 and Trp183 are important for its folding and oxidase function. Biosci Rep. 2015;35: pubmed publisher
    b>Erv1 is an FAD-dependent thiol oxidase of the ERV (essential for respiration and viability)/ALR (augmenter of liver regeneration) sub-family and an essential component of the mitochondrial import and assembly pathway...
  12. Ramesh A, Peleh V, Martinez Caballero S, Wollweber F, Sommer F, van der Laan M, et al. A disulfide bond in the TIM23 complex is crucial for voltage gating and mitochondrial protein import. J Cell Biol. 2016;214:417-31 pubmed publisher
    ..Our observations suggest that Tim17 can be directly oxidized by the sulfhydryl oxidase Erv1. Thus, import and oxidation of Tim17 are mediated by the mitochondrial disulfide relay, though the mechanism by ..
  13. Wrobel L, Trojanowska A, Sztolsztener M, Chacinska A. Mitochondrial protein import: Mia40 facilitates Tim22 translocation into the inner membrane of mitochondria. Mol Biol Cell. 2013;24:543-54 pubmed publisher
    ..We propose that Mia40 not only is responsible for disulfide bond formation, but also assists the Tim22 protein in its integration into the inner membrane of mitochondria. ..
  14. Neal S, Dabir D, Wijaya J, Boon C, Koehler C. Osm1 facilitates the transfer of electrons from Erv1 to fumarate in the redox-regulated import pathway in the mitochondrial intermembrane space. Mol Biol Cell. 2017;28:2773-2785 pubmed publisher
    ..The mitochondrial intermembrane space has an analogous pathway with the oxidoreductase Mia40 and sulfhydryl oxidase Erv1, termed the mitochondrial intermembrane space assembly (MIA) pathway...
  15. Mesecke N, Bihlmaier K, Grumbt B, Longen S, Terziyska N, Hell K, et al. The zinc-binding protein Hot13 promotes oxidation of the mitochondrial import receptor Mia40. EMBO Rep. 2008;9:1107-13 pubmed publisher
    ..This system consists of two essential proteins, Mia40 and Erv1, which bind to newly imported proteins by disulphide transfer...
  16. Morgan B, Ang S, Yan G, Lu H. Zinc can play chaperone-like and inhibitor roles during import of mitochondrial small Tim proteins. J Biol Chem. 2009;284:6818-25 pubmed publisher
    ..Furthermore, our results show that zinc is a powerful inhibitor of Erv1, an essential component of the import pathway used by the small Tim proteins...
  17. Ang S, Zhang M, Lodi T, Lu H. Mitochondrial thiol oxidase Erv1: both shuttle cysteine residues are required for its function with distinct roles. Biochem J. 2014;460:199-210 pubmed publisher
    b>Erv1 (essential for respiration and viability 1), is an essential component of the MIA (mitochondrial import and assembly) pathway, playing an important role in the oxidative folding of mitochondrial intermembrane space proteins...
  18. Neal S, Dabir D, Tienson H, Horn D, Glaeser K, Ogozalek Loo R, et al. Mia40 Protein Serves as an Electron Sink in the Mia40-Erv1 Import Pathway. J Biol Chem. 2015;290:20804-14 pubmed publisher
    A redox-regulated import pathway consisting of Mia40 and Erv1 mediates the import of cysteine-rich proteins into the mitochondrial intermembrane space...
  19. Ozer H, Dlouhy A, Thornton J, Hu J, Liu Y, Barycki J, et al. Cytosolic Fe-S Cluster Protein Maturation and Iron Regulation Are Independent of the Mitochondrial Erv1/Mia40 Import System. J Biol Chem. 2015;290:27829-40 pubmed publisher
    The sulfhydryl oxidase Erv1 partners with the oxidoreductase Mia40 to import cysteine-rich proteins in the mitochondrial intermembrane space...