Gene Symbol: OXA1
Description: membrane insertase OXA1
Alias: membrane insertase OXA1
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Szyrach G, Ott M, Bonnefoy N, Neupert W, Herrmann J. Ribosome binding to the Oxa1 complex facilitates co-translational protein insertion in mitochondria. EMBO J. 2003;22:6448-57 pubmed
    The Oxa1 translocase of the mitochondrial inner membrane facilitates the insertion of both mitochondrially and nuclear-encoded proteins from the matrix into the inner membrane...
  2. Hell K, Herrmann J, Pratje E, Neupert W, Stuart R. Oxa1p, an essential component of the N-tail protein export machinery in mitochondria. Proc Natl Acad Sci U S A. 1998;95:2250-5 pubmed
    ..Thus, Oxa1p represents a component of a general export machinery of the mitochondrial inner membrane. ..
  3. Reif S, Randelj O, Domanska G, Dian E, Krimmer T, Motz C, et al. Conserved mechanism of Oxa1 insertion into the mitochondrial inner membrane. J Mol Biol. 2005;354:520-8 pubmed
    b>Oxa1 is the mitochondrial representative of a family of related proteins that mediate the insertion of substrate proteins into the membranes of bacteria, chloroplasts, and mitochondria...
  4. He S, Fox T. Membrane translocation of mitochondrially coded Cox2p: distinct requirements for export of N and C termini and dependence on the conserved protein Oxa1p. Mol Biol Cell. 1997;8:1449-60 pubmed
    ..The mitochondrial export system does not closely resemble the bacterial Sec translocase. However, normal translocation of both exported domains of Cox2p was defective in cells lacking the widely conserved inner membrane protein Oxa1p. ..
  5. Hell K, Neupert W, Stuart R. Oxa1p acts as a general membrane insertion machinery for proteins encoded by mitochondrial DNA. EMBO J. 2001;20:1281-8 pubmed
    Oxa1p is a member of the conserved Oxa1/YidC/Alb3 protein family involved in the membrane insertion of proteins...
  6. Meyer W, Bauer M, Pratje E. A mutation in cytochrome oxidase subunit 2 restores respiration of the mutant pet ts1402. Curr Genet. 1997;31:401-7 pubmed
    The yeast PET1402/OXA1 gene encoding a 44.8-kDa protein is required for mitochondrial biogenesis...
  7. Fiumera H, Dunham M, Saracco S, Butler C, Kelly J, Fox T. Translocation and assembly of mitochondrially coded Saccharomyces cerevisiae cytochrome c oxidase subunit Cox2 by Oxa1 and Yme1 in the absence of Cox18. Genetics. 2009;182:519-28 pubmed publisher
    Members of the Oxa1/YidC/Alb3 family of protein translocases are essential for assembly of energy-transducing membrane complexes...
  8. Hamel P, Lemaire C, Bonnefoy N, Brivet Chevillotte P, Dujardin G. Mutations in the membrane anchor of yeast cytochrome c1 compensate for the absence of Oxa1p and generate carbonate-extractable forms of cytochrome c1. Genetics. 1998;150:601-11 pubmed
    ..We propose that these carbonate-extractable forms of cytochrome c1 are responsible for the suppressor function by preventing the degradation of the respiratory complex subunits that occur in the absence of Oxa1p. ..
  9. Rep M, Nooy J, Guelin E, Grivell L. Three genes for mitochondrial proteins suppress null-mutations in both Afg3 and Rca1 when over-expressed. Curr Genet. 1996;30:206-11 pubmed
    ..PIM1(LON) encodes a matrix-localized ATP-dependent protease involved in the turnover of matrix proteins; OXA1(PET1402) encodes a putative mitochondrial inner membrane protein involved in the biogenesis of the respiratory ..

More Information


  1. Jia L, Dienhart M, Stuart R. Oxa1 directly interacts with Atp9 and mediates its assembly into the mitochondrial F1Fo-ATP synthase complex. Mol Biol Cell. 2007;18:1897-908 pubmed
    The yeast Oxa1 protein is involved in the biogenesis of the mitochondrial oxidative phosphorylation (OXPHOS) machinery...
  2. Hell K, Herrmann J, Pratje E, Neupert W, Stuart R. Oxa1p mediates the export of the N- and C-termini of pCoxII from the mitochondrial matrix to the intermembrane space. FEBS Lett. 1997;418:367-70 pubmed
    ..In conclusion, Oxa1p mediates the export of the N- and C-termini of the mitochondrially encoded subunit II of cytochrome oxidase from the matrix to the intermembrane space. ..
  3. Herrmann J, Neupert W, Stuart R. Insertion into the mitochondrial inner membrane of a polytopic protein, the nuclear-encoded Oxa1p. EMBO J. 1997;16:2217-26 pubmed
    ..We postulate that at least two different sorting pathways exist in mitochondria for polytopic inner membrane proteins, the evolutionarily novel pathway for members of the ADP/ATP carrier family and a conserved Oxa1p-type pathway. ..
  4. Jia L, Dienhart M, Schramp M, McCauley M, Hell K, Stuart R. Yeast Oxa1 interacts with mitochondrial ribosomes: the importance of the C-terminal region of Oxa1. EMBO J. 2003;22:6438-47 pubmed
    The yeast mitochondrial Oxa1 protein is a member of the conserved Oxa1/YidC/Alb3 protein family involved in the membrane insertion of proteins...
  5. Hallstrom T, Moye Rowley W. Multiple signals from dysfunctional mitochondria activate the pleiotropic drug resistance pathway in Saccharomyces cerevisiae. J Biol Chem. 2000;275:37347-56 pubmed
    ..Importantly, the signaling pathway used to enhance Pdr3p function in rho(o) cells is not the same as in oxa1 cells...
  6. Slobodin B, Gerst J. A novel mRNA affinity purification technique for the identification of interacting proteins and transcripts in ribonucleoprotein complexes. RNA. 2010;16:2277-90 pubmed publisher
    ..g., ASH1-She2; ?-Actin-IMP1). By isolating tagged OXA1 mRNA using RaPID, we could identify a yeast COPI subunit (i.e., Sec27) as a candidate interacting protein...
  7. Nebauer R, Schuiki I, Kulterer B, Trajanoski Z, Daum G. The phosphatidylethanolamine level of yeast mitochondria is affected by the mitochondrial components Oxa1p and Yme1p. FEBS J. 2007;274:6180-90 pubmed
    ..Here, we report that deletion of OXA1 encoding an inner mitochondrial membrane protein translocase markedly affects the mitochondrial ..
  8. Liu Y, Wang X, Chen X. Misfolding of mutant adenine nucleotide translocase in yeast supports a novel mechanism of Ant1-induced muscle diseases. Mol Biol Cell. 2015;26:1985-94 pubmed publisher
    ..This finding could have broad implications for understanding other dominant diseases (e.g., retinitis pigmentosa) caused by missense mutations in membrane proteins. ..
  9. Preuss M, Leonhard K, Hell K, Stuart R, Neupert W, Herrmann J. Mba1, a novel component of the mitochondrial protein export machinery of the yeast Saccharomyces cerevisiae. J Cell Biol. 2001;153:1085-96 pubmed
    ..The inner membrane protein Oxa1 plays an important role in this process...
  10. Saint Georges Y, Hamel P, Lemaire C, Dujardin G. Role of positively charged transmembrane segments in the insertion and assembly of mitochondrial inner-membrane proteins. Proc Natl Acad Sci U S A. 2001;98:13814-9 pubmed
    ..This model argues in favor of a role of Oxa1p in the insertion and the lateral exit of less hydrophobic TM domains from the translocation site into the lipid bilayer. ..
  11. Kohler R, Boehringer D, Greber B, Bingel Erlenmeyer R, Collinson I, Schaffitzel C, et al. YidC and Oxa1 form dimeric insertion pores on the translating ribosome. Mol Cell. 2009;34:344-53 pubmed publisher
    The YidC/Oxa1/Alb3 family of membrane proteins facilitates the insertion and assembly of membrane proteins in bacteria, mitochondria, and chloroplasts...
  12. Mokranjac D, Sichting M, Popov Celeketic D, Mapa K, Gevorkyan Airapetov L, Zohary K, et al. Role of Tim50 in the transfer of precursor proteins from the outer to the inner membrane of mitochondria. Mol Biol Cell. 2009;20:1400-7 pubmed publisher
    ..This function of Tim50 depends on its association with TIM23. We conclude that the efficient transfer of precursors between TOM and TIM23 complexes requires the concerted action of Tim50 with Tim23. ..
  13. Mathieu L, Bourens M, Marsy S, Hlavacek O, Panozzo C, Dujardin G. A mutational analysis reveals new functional interactions between domains of the Oxa1 protein in Saccharomyces cerevisiae. Mol Microbiol. 2010;75:474-88 pubmed publisher
    The Oxa1/YidC/Alb3 family plays a key role in the biogenesis of the respiratory and photosynthetic complexes in bacteria and organelles...
  14. Funes S, Hasona A, Bauerschmitt H, Grubbauer C, Kauff F, Collins R, et al. Independent gene duplications of the YidC/Oxa/Alb3 family enabled a specialized cotranslational function. Proc Natl Acad Sci U S A. 2009;106:6656-61 pubmed publisher
    ..This is reminiscent of the situation in mitochondria that lack an SRP pathway and where Oxa1 facilitates cotranslational membrane protein insertion by binding directly to translation-active ribosomes...
  15. Keil M, Bareth B, Woellhaf M, Peleh V, Prestele M, Rehling P, et al. Oxa1-ribosome complexes coordinate the assembly of cytochrome C oxidase in mitochondria. J Biol Chem. 2012;287:34484-93 pubmed publisher
    ..on mitochondrial ribosomes and inserted into the inner membrane in a co-translational reaction facilitated by the Oxa1 insertase. Oxa1 consists of an N-terminal insertase domain and a C-terminal ribosome-binding region...
  16. Iacovino M, Granycome C, Sembongi H, Bokori Brown M, Butow R, Holt I, et al. The conserved translocase Tim17 prevents mitochondrial DNA loss. Hum Mol Genet. 2009;18:65-74 pubmed publisher
    ..For example, Ilv5 is required for branched chain amino acid biosynthesis and mtDNA stability. We have identified OXA1 and TIM17 as novel multicopy suppressors of mtDNA instability in ilv5 cells...
  17. Glatigny A, Mathieu L, Herbert C, Dujardin G, Meunier B, Mucchielli Giorgi M. An in silico approach combined with in vivo experiments enables the identification of a new protein whose overexpression can compensate for specific respiratory defects in Saccharomyces cerevisiae. BMC Syst Biol. 2011;5:173 pubmed publisher
    ..Thus, this systems biology approach appears to be a fruitful new way to study the biogenesis of mitochondrial multi-subunit complexes. ..
  18. Jia L, Kaur J, Stuart R. Mapping of the Saccharomyces cerevisiae Oxa1-mitochondrial ribosome interface and identification of MrpL40, a ribosomal protein in close proximity to Oxa1 and critical for oxidative phosphorylation complex assembly. Eukaryot Cell. 2009;8:1792-802 pubmed publisher
    The Oxa1 protein plays a central role in facilitating the cotranslational insertion of the nascent polypeptide chains into the mitochondrial inner membrane...
  19. Ott M, Prestele M, Bauerschmitt H, Funes S, Bonnefoy N, Herrmann J. Mba1, a membrane-associated ribosome receptor in mitochondria. EMBO J. 2006;25:1603-10 pubmed
    ..It thereby cooperates with the C-terminal ribosome-binding domain of Oxa1, which is a central component of the insertion machinery of the inner membrane...
  20. Gadir N, Haim Vilmovsky L, Kraut Cohen J, Gerst J. Localization of mRNAs coding for mitochondrial proteins in the yeast Saccharomyces cerevisiae. RNA. 2011;17:1551-65 pubmed publisher
    ..We examined the specific determinants of ATP2 and OXA1 mRNA localization and found a mutual dependency on the 3' UTR, Puf3, Tom7, and Tom70, but not Tom20, for ..
  21. Bauerschmitt H, Funes S, Herrmann J. The membrane-bound GTPase Guf1 promotes mitochondrial protein synthesis under suboptimal conditions. J Biol Chem. 2008;283:17139-46 pubmed publisher
    ..Moreover, Guf1 mutants exhibit synthetic growth defects with mutations of the protein insertase Oxa1. These observations show a critical role for Guf1 in vivo...
  22. Stoldt S, Wenzel D, Hildenbeutel M, Wurm C, Herrmann J, Jakobs S. The inner-mitochondrial distribution of Oxa1 depends on the growth conditions and on the availability of substrates. Mol Biol Cell. 2012;23:2292-301 pubmed publisher
    The Oxa1 protein is a well-conserved integral protein of the inner membrane of mitochondria. It mediates the insertion of both mitochondrial- and nuclear-encoded proteins from the matrix into the inner membrane...
  23. Hlavacek O, Bourens M, Salone V, Lachacinski N, Lemaire C, Dujardin G. The transcriptional activator HAP4 is a high copy suppressor of an oxa1 yeast mutation. Gene. 2005;354:53-7 pubmed
    ..for the insertion of membrane proteins in mitochondria, and in the yeast Saccharomyces cerevisiae, the deletion of OXA1 impairs the biogenesis of the three respiratory complexes of dual genetic origin...
  24. Fiumera H, Broadley S, Fox T. Translocation of mitochondrially synthesized Cox2 domains from the matrix to the intermembrane space. Mol Cell Biol. 2007;27:4664-73 pubmed
    ..the Cox2 C-terminal domain were translocated to the IMS via a pathway dependent upon the conserved translocase Oxa1. The topology of this Cox2 variant, accumulated at steady state, was largely but not completely unaffected in ..
  25. Kr├╝ger V, Deckers M, Hildenbeutel M, van der Laan M, Hellmers M, Dreker C, et al. The mitochondrial oxidase assembly protein1 (Oxa1) insertase forms a membrane pore in lipid bilayers. J Biol Chem. 2012;287:33314-26 pubmed
    ..b>Oxa1, a conserved membrane protein, mediates the insertion of mitochondrion-encoded precursors into the inner ..
  26. Lemaire C, Guibet Grandmougin F, Angles D, Dujardin G, Bonnefoy N. A yeast mitochondrial membrane methyltransferase-like protein can compensate for oxa1 mutations. J Biol Chem. 2004;279:47464-72 pubmed
    ..A conserved motif within this domain is essential for the suppression of oxa1 mutations. We discuss the possible role of Oms1p on Oxa1p intermembrane space domain.
  27. Funes S, Westerburg H, Jaimes Miranda F, Woellhaf M, Aguilar Lopez J, Jansen L, et al. Partial suppression of Oxa1 mutants by mitochondria-targeted signal recognition particle provides insights into the evolution of the cotranslational insertion systems. FEBS J. 2013;280:904-15 pubmed publisher
    ..Accordingly, deletion of ribosome-binding sites on the Oxa1 insertase and the Mba1 ribosome receptor in yeast leads to severe defects in cotranslational protein insertion and ..
  28. Hildenbeutel M, Theis M, Geier M, Haferkamp I, Neuhaus H, Herrmann J, et al. The membrane insertase Oxa1 is required for efficient import of carrier proteins into mitochondria. J Mol Biol. 2012;423:590-9 pubmed publisher
    b>Oxa1 serves as a protein insertase of the mitochondrial inner membrane that is evolutionary related to the bacterial YidC insertase...