Gene Symbol: YME1
Description: i-AAA protease YME1
Alias: OSD1, YTA11, i-AAA protease YME1
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Weber E, Rooks R, Shafer K, Chase J, Thorsness P. Mutations in the mitochondrial ATP synthase gamma subunit suppress a slow-growth phenotype of yme1 yeast lacking mitochondrial DNA. Genetics. 1995;140:435-42 pubmed
    In Saccharomyces cerevisiae, inactivation of the nuclear gene YME1 causes several phenotypes associated with impairment of mitochondrial function...
  2. Rainey R, Glavin J, Chen H, French S, Teitell M, Koehler C. A new function in translocation for the mitochondrial i-AAA protease Yme1: import of polynucleotide phosphorylase into the intermembrane space. Mol Cell Biol. 2006;26:8488-97 pubmed
    ..The i-AAA protease Yme1 mediated translocation of PNPase into the intermembrane space but did not degrade PNPase...
  3. Hwang D, Claypool S, Leuenberger D, Tienson H, Koehler C. Tim54p connects inner membrane assembly and proteolytic pathways in the mitochondrion. J Cell Biol. 2007;178:1161-75 pubmed
    ..As such, Tim54p links protein import, assembly, and turnover pathways in the mitochondrion. ..
  4. Graef M, Seewald G, Langer T. Substrate recognition by AAA+ ATPases: distinct substrate binding modes in ATP-dependent protease Yme1 of the mitochondrial intermembrane space. Mol Cell Biol. 2007;27:2476-85 pubmed
    ..steps of substrate recognition and identify two distinct substrate binding sites in the i-AAA protease subunit Yme1. Misfolded polypeptides are recognized by conserved helices in proteolytic and AAA domains...
  5. Kominsky D, Brownson M, Updike D, Thorsness P. Genetic and biochemical basis for viability of yeast lacking mitochondrial genomes. Genetics. 2002;162:1595-604 pubmed
    ..F(1)F(0)-ATPase activity is reduced in yeast lacking Yme1p and is restored in yme1 strains bearing suppressing mutations in F(1)-ATPase structural genes...
  6. Dunn C, Lee M, Spencer F, Jensen R. A genomewide screen for petite-negative yeast strains yields a new subunit of the i-AAA protease complex. Mol Biol Cell. 2006;17:213-26 pubmed
    ..Our results highlight the importance of the i-AAA complex and proteolysis at the inner membrane in cells lacking mitochondrial DNA. ..
  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
    ..While Mgr1 and Mgr3 are known to associate with the Yme1 mitochondrial inner membrane i-AAA protease and to participate in membrane protein degradation, their absence does ..
  8. Weber E, Hanekamp T, Thorsness P. Biochemical and functional analysis of the YME1 gene product, an ATP and zinc-dependent mitochondrial protease from S. cerevisiae. Mol Biol Cell. 1996;7:307-17 pubmed
    Inactivation of YME1 in yeast causes several distinct phenotypes: an increased rate of DNA escape from mitochondria, temperature-sensitive growth on nonfermentable carbon sources, extremely slow growth when mitochondrial DNA is completely ..
  9. Francis B, White K, Thorsness P. Mutations in the Atp1p and Atp3p subunits of yeast ATP synthase differentially affect respiration and fermentation in Saccharomyces cerevisiae. J Bioenerg Biomembr. 2007;39:127-44 pubmed
    ..Spore viability of strains bearing ATP1-111 is reduced compared to wild type, although ATP1-111 enhances the survival of spores that lacked mitochondrial DNA. ..

More Information


  1. Potting C, Wilmes C, Engmann T, Osman C, Langer T. Regulation of mitochondrial phospholipids by Ups1/PRELI-like proteins depends on proteolysis and Mdm35. EMBO J. 2010;29:2888-98 pubmed publisher
    ..The turnover of Ups2 is mediated by the i-AAA protease Yme1, whereas Ups1 is degraded by both Yme1 and the metallopeptidase Atp23...
  2. Bestwick M, Khalimonchuk O, Pierrel F, Winge D. The role of Coa2 in hemylation of yeast Cox1 revealed by its genetic interaction with Cox10. Mol Cell Biol. 2010;30:172-85 pubmed publisher
    ..We postulate that the mutant Cox10 complex has enhanced efficiency in the addition of heme a to Cox1. Coa2 appears to impart stability to the oligomeric wild-type Cox10 complex involved in Cox1 hemylation. ..
  3. Baker M, Mooga V, Guiard B, Langer T, Ryan M, Stojanovski D. Impaired folding of the mitochondrial small TIM chaperones induces clearance by the i-AAA protease. J Mol Biol. 2012;424:227-39 pubmed publisher
    ..We delineate a clearance mechanism for the mutant proteins and their unassembled wild-type partner protein by the mitochondrial ATP-dependent protease, Yme1 (yeast mitochondrial escape 1).
  4. Elliott L, Saracco S, Fox T. Multiple roles of the Cox20 chaperone in assembly of Saccharomyces cerevisiae cytochrome c oxidase. Genetics. 2012;190:559-67 pubmed publisher
    ..The requirement for Cox20 in cytochrome c oxidase assembly and respiratory growth is partially bypassed by yme1, mgr1 or mgr3 mutations, each of which reduce i-AAA protease activity in the intermembrane space...
  5. 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
    ..In summary, our results demonstrate a link between the mitochondrial protein import machinery, assembly and stability of Psd1p, and phosphatidylethanolamine homeostasis in yeast mitochondria. ..
  6. Kambacheld M, Augustin S, Tatsuta T, Muller S, Langer T. Role of the novel metallopeptidase Mop112 and saccharolysin for the complete degradation of proteins residing in different subcompartments of mitochondria. J Biol Chem. 2005;280:20132-9 pubmed
    ..These results suggest that the turnover of mitochondrial proteins is mediated by the sequential action of ATP-dependent proteases and oligopeptidases, some of them localized in the intermembrane space. ..
  7. Dunn C, Tamura Y, Sesaki H, Jensen R. Mgr3p and Mgr1p are adaptors for the mitochondrial i-AAA protease complex. Mol Biol Cell. 2008;19:5387-97 pubmed publisher
    ..We speculate that Mgr3p and Mgr1p function in an adaptor complex that targets substrates to the i-AAA protease for degradation. ..
  8. Wang K, Jin M, Liu X, Klionsky D. Proteolytic processing of Atg32 by the mitochondrial i-AAA protease Yme1 regulates mitophagy. Autophagy. 2013;9:1828-36 pubmed publisher
    ..Furthermore, we determined that the mitochondrial i-AAA protease Yme1 mediated Atg32 processing and was required for mitophagy...
  9. Campbell C, Tanaka N, White K, Thorsness P. Mitochondrial morphological and functional defects in yeast caused by yme1 are suppressed by mutation of a 26S protease subunit homologue. Mol Biol Cell. 1994;5:899-905 pubmed
    ..This proteolytic activity is necessary for progression through the cell cycle and has been implicated in the regulation of transcription. Ynt1p is more distantly related to Yme1p. ..
  10. Garipler G, Mutlu N, Lack N, Dunn C. Deletion of conserved protein phosphatases reverses defects associated with mitochondrial DNA damage in Saccharomyces cerevisiae. Proc Natl Acad Sci U S A. 2014;111:1473-8 pubmed publisher
    ..Our work highlights the important role that nutrient-responsive signaling pathways can play in determining the response to mitochondrial dysfunction. ..
  11. Dasari S, Kölling R. Role of mitochondrial processing peptidase and AAA proteases in processing of the yeast acetohydroxyacid synthase precursor. FEBS Open Bio. 2016;6:765-73 pubmed publisher
    ..Both, precursor to A-form and A-form to B-form cleavage were strongly affected in a ?yme1 mutant...
  12. Huang Z, Chen K, Xu T, Zhang J, Li Y, Li W, et al. Sampangine inhibits heme biosynthesis in both yeast and human. Eukaryot Cell. 2011;10:1536-44 pubmed publisher
    ..This study also reveals a surprising essential role for the interaction between the mitochondrial ATP synthase and the electron transport chain. ..
  13. Hanekamp T, Thorsness P. Inactivation of YME2/RNA12, which encodes an integral inner mitochondrial membrane protein, causes increased escape of DNA from mitochondria to the nucleus in Saccharomyces cerevisiae. Mol Cell Biol. 1996;16:2764-71 pubmed
    ..Mutations in yme2 also show genetic interactions with yme1, a second gene that affects DNA escape from mitochondria to the nucleus...
  14. Chen X, Moerschell R, Pearce D, Ramanan D, Sherman F. Enhanced mitochondrial degradation of yeast cytochrome c with amphipathic structures. Curr Genet. 2005;47:67-83 pubmed
    ..Most importantly, ADD appeared to be specifically suppressed to various extents by deletions of any of the YME1, AFG3, or RCA1 genes encoding membrane-associated mitochondrial proteases, probably because the amphipathic ..
  15. Hanekamp T, Thorsness P. YNT20, a bypass suppressor of yme1 yme2, encodes a putative 3'-5' exonuclease localized in mitochondria of Saccharomyces cerevisiae. Curr Genet. 1999;34:438-48 pubmed
    ..The synthetic respiratory growth defect of yme1 yme2 yeast strains is suppressed by recessive mutations in YNT20...
  16. Lemaire C, Hamel P, Velours J, Dujardin G. Absence of the mitochondrial AAA protease Yme1p restores F0-ATPase subunit accumulation in an oxa1 deletion mutant of Saccharomyces cerevisiae. J Biol Chem. 2000;275:23471-5 pubmed
    ..In addition, although respiratory function is dispensable in Saccharomyces cerevisiae, we show that the simultaneous inactivation of AFG3 and YME1 is lethal and that the essential function does not reside in their protease activity.
  17. Puchades C, Rampello A, Shin M, Giuliano C, Wiseman R, Glynn S, et al. Structure of the mitochondrial inner membrane AAA+ protease YME1 gives insight into substrate processing. Science. 2017;358: pubmed publisher
    We present an atomic model of a substrate-bound inner mitochondrial membrane AAA+ quality control protease in yeast, YME1. Our ~3...
  18. Wang X, Zuo X, Kucejova B, Chen X. Reduced cytosolic protein synthesis suppresses mitochondrial degeneration. Nat Cell Biol. 2008;10:1090-7 pubmed
    ..Our finding thus establishes a link between protein homeostasis (proteostasis), cellular bioenergetics and mitochondrial maintenance during ageing. ..
  19. Ogunbona O, Onguka O, Calzada E, Claypool S. Multitiered and Cooperative Surveillance of Mitochondrial Phosphatidylserine Decarboxylase 1. Mol Cell Biol. 2017;37: pubmed publisher
  20. Spiller M, Guo L, Wang Q, Tran P, Lu H. Mitochondrial Tim9 protects Tim10 from degradation by the protease Yme1. Biosci Rep. 2015;35: pubmed publisher
    ..be suppressed by deletion of the mitochondrial i-AAA (ATPases associated with diverse cellular activities) protease Yme1, and this correlates strongly with stabilization of the Tim10 protein regardless of Tim9 levels...
  21. Bohovych I, Donaldson G, Christianson S, Zahayko N, Khalimonchuk O. Stress-triggered activation of the metalloprotease Oma1 involves its C-terminal region and is important for mitochondrial stress protection in yeast. J Biol Chem. 2014;289:13259-72 pubmed publisher
    ..These findings indicate that yeast Oma1 is an important player in IM protein homeostasis and integrity by acting in concert with other intramitochondrial quality control components. ..
  22. Schreiner B, Westerburg H, Forne I, Imhof A, Neupert W, Mokranjac D. Role of the AAA protease Yme1 in folding of proteins in the intermembrane space of mitochondria. Mol Biol Cell. 2012;23:4335-46 pubmed publisher
    ..b>Yme1, an AAA (ATPases associated with diverse cellular activities) protease of the IMS, prevented aggregation of DHFR...
  23. Palermo V, Falcone C, Mazzoni C. Apoptosis and aging in mitochondrial morphology mutants of S. cerevisiae. Folia Microbiol (Praha). 2007;52:479-83 pubmed
    ..In contrast, YME1, encoding a subunit of the mitochondrial inner membrane i-AAA proteinase complex, has a protective role in these ..
  24. Claypool S, Whited K, Srijumnong S, Han X, Koehler C. Barth syndrome mutations that cause tafazzin complex lability. J Cell Biol. 2011;192:447-62 pubmed publisher
    ..Thus, the loss of function for these BTHS mutants results from the inherent instability of the mutant tafazzin complexes. ..
  25. Rampello A, Glynn S. Identification of a Degradation Signal Sequence within Substrates of the Mitochondrial i-AAA Protease. J Mol Biol. 2017;429:873-885 pubmed publisher
    ..Together, these results identify the first specific degron sequence within a native i-AAA protease substrate. ..
  26. Heo J, Livnat Levanon N, Taylor E, Jones K, Dephoure N, Ring J, et al. A stress-responsive system for mitochondrial protein degradation. Mol Cell. 2010;40:465-80 pubmed publisher
    ..We demonstrate that Vms1 is a required component of an evolutionarily conserved system for mitochondrial protein degradation, which is necessary to maintain mitochondrial, cellular, and organismal viability. ..
  27. Gaspard G, McMaster C. The mitochondrial quality control protein Yme1 is necessary to prevent defective mitophagy in a yeast model of Barth syndrome. J Biol Chem. 2015;290:9284-98 pubmed publisher
    ..Focusing on the i-AAA protease Yme1, a mitochondrial quality control protein that degrades misfolded proteins, we determined that in cells lacking both ..
  28. Wang X, Salinas K, Zuo X, Kucejova B, Chen X. Dominant membrane uncoupling by mutant adenine nucleotide translocase in mitochondrial diseases. Hum Mol Genet. 2008;17:4036-44 pubmed publisher
    ..mtDNA disintegration is a phenotype co-lateral to mitochondrial damages. These findings provide mechanistic insights into the pathogenesis of the Ant1-induced diseases. ..
  29. Francis B, Thorsness P. Hsp90 and mitochondrial proteases Yme1 and Yta10/12 participate in ATP synthase assembly in Saccharomyces cerevisiae. Mitochondrion. 2011;11:587-600 pubmed publisher
    ..Inactivation of either of the mitochondrial AAA proteases, Yme1 or Yta10/12, allows fermentative growth of hsc82? or hsp82? strains at 37°C...
  30. 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. ..
  31. Thorsness P, Fox T. Nuclear mutations in Saccharomyces cerevisiae that affect the escape of DNA from mitochondria to the nucleus. Genetics. 1993;134:21-8 pubmed
    ..All 21 mutations were recessive and fell into six complementation groups, termed YME1-YME6...