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References

  • 1
    Sickmann, A., Reinders, J., Wagner, Y., Joppich, C., Zahedi, R., etal. ( 2003) The proteome of Saccharomyces cerevisiae mitochondria. Proc. Natl. Acad. Sci. USA 100, 1320713212.
  • 2
    Dolezal, P., Likic, V., Tachezy, J., and Lithgow, T. ( 2006) Evolution of the molecular machines for protein import into mitochondria. Science 313, 314318.
  • 3
    Pagliarini, D. J., Calvo, S. E., Chang, B., Sheth, S. A., Vafai, S. B., etal. ( 2008) A mitochondrial protein compendium elucidates complex I disease biology. Cell 134, 112123.
  • 4
    Neupert, W. and Herrmann, J. M. ( 2007) Translocation of proteins into mitochondria. Annu. Rev. Biochem. 76, 723749.
  • 5
    Chacinska, A., Koehler, C. M., Milenkovic, D., Lithgow, T., and Pfanner, N. ( 2009) Importing mitochondrial proteins: machineries and mechanisms. Cell 138, 628644.
  • 6
    Schmidt, O., Pfanner, N., and Meisinger, C. ( 2010) Mitochondrial protein import: from proteomics to functional mechanisms. Nat. Rev. Mol. Cell Biol. 11, 655667.
  • 7
    Becker, T., Böttinger, L., and Pfanner, N. ( 2012) Mitochondrial protein import: from transport pathways to an integrated network. Trends Biochem. Sci. 37, 8591.
  • 8
    Schmidt, O., Harbauer, A. B., Rao, S., Eyrich, B., Zahedi, R. P., etal. ( 2011) Regulation of mitochondrial protein import by cytosolic kinases. Cell 144, 227239.
  • 9
    Rao, S., Schmidt, O., Harbauer, A. B., Schönfisch, B., Guiard, B., etal. ( 2012) Biogenesis of the preprotein translocase of the outer mitochondrial membrane: protein kinase A phosphorylates the precursor of Tom40 and impairs its import. Mol. Biol. Cell 23, 16181627.
  • 10
    Young, J. C., Hoogenraad, N. J., and Hartl, F.U. ( 2003) Molecular chaperones Hsp90 and Hsp70 deliver preproteins to the mitochondrial import receptor Tom70. Cell 112, 4150.
  • 11
    Zara, V., Ferramosca, A., Robitaille-Foucher, P., Palmieri, F., and Young, J. C. ( 2009) Mitochondrial carrier protein biogenesis: role of the chaperones Hsc70 and Hsp90. Biochem. J. 419, 369375.
  • 12
    Kellems, R. E., Allison, V. F., and Butow, R. A. ( 1975) Cytoplasmic type 80S ribosomes associated with yeast mitochondria. IV. Attachment of ribosomes to the outer membrane of isolated mitochondria. J. Cell Biol. 65, 114.
  • 13
    Suissa, M. and Schatz, G. ( 1982) Import of proteins into mitochondria. Translatable mRNAs for imported mitochondrial proteins are present in free as well as mitochondria-bound cytoplasmic polysomes. J. Biol. Chem. 257, 1304813055.
  • 14
    Marc, P., Margeot, A., Devaux, F., Blugeon, C., Corral-Debrinski, M., etal. ( 2002) Genome-wide analysis of mRNAs targeted to yeast mitochondria. EMBO Rep. 3, 159164.
  • 15
    Garcia, M., Darzacq, X., Devaux, F., Singer, R.H., and Jacq, C. ( 2007) Yeast mitochondrial transcriptomics. Methods Mol. Biol. 372, 505528.
  • 16
    Saint-Georges, Y., Garcia, M., Delaveau, T., Jourdren, L., Le Crom, S., etal. ( 2008) Yeast mitochondrial biogenesis: a role for the PUF RNA-binding protein Puf3p in mRNA localization. PLoS One 3, e2293.
  • 17
    Eliyahu, E., Pnueli, L., Melamed, D., Scherrer, T., Gerber, A. P., etal. ( 2010) Tom20 mediates localization of mRNAs to mitochondria in a translation-dependent manner. Mol. Cell. Biol. 30, 284294.
  • 18
    Quenault, T., Lithgow, T., and Traven, A. ( 2011) PUF proteins: repression, activation and mRNA localization. Trends Cell Biol. 21, 104112.
  • 19
    Yogev, O., Karniely, S., and Pines, O. ( 2007) Translation-coupled translocation of yeast fumarase into mitochondria in vivo. J. Biol. Chem. 282, 2922229229.
  • 20
    Luk, E., Yang, M., Jensen, L. T., Bourbonnais, Y., and Culotta, V. C. ( 2005) Manganese activation of superoxide dismutase 2 in the mitochondria of Saccharomyces cerevisiae. J. Biol. Chem. 280, 2271522720.
  • 21
    Fünfschilling, U. and Rospert, S. ( 1999) Nascent polypeptide-associated complex stimulates protein import into yeast mitochondria. Mol. Biol. Cell 10, 32893299.
  • 22
    Isken, O. and Maquat, L. E. ( 2007) Quality control of eukaryotic mRNA: safeguarding cells from abnormal mRNA function. Genes Dev. 21, 18331856.
  • 23
    Izawa, T., Tsuboi, T., Kuroha, K., Inada, T., Nishikawa, S., etal. ( 2012) Roles of dom34:hbs1 in nonstop protein clearance from translocators for normal organelle protein influx. Cell Rep. 2, 447453.
  • 24
    Dudek, J., Rehling, P., and van der Laan, M. Mitochondrial protein import: common principles and physiological networks. Biochim. Biophys. Acta, DOI:10.1016/j.bbamcr.2012.05.028.
  • 25
    Mokranjac, D. and Neupert, W. ( 2010) The many faces of the mitochondrial TIM23 complex. Biochim. Biophys. Acta 1797, 10451054.
  • 26
    Mokranjac, D., Sichting, M., Popov-Celeketić, D., Mapa, K., Gevorkyan-Airapetov, L., etal. ( 2009) Role of Tim50 in the transfer of precursor proteins from the outer to the inner membrane of mitochondria. Mol. Biol. Cell 20, 14001407.
  • 27
    Marom, M., Dayan, D., Demishtein-Zohary, K., Mokranjac, D., Neupert, W., etal. ( 2011) Direct interaction of mitochondrial targeting presequences with purified components of the TIM23 protein complex. J. Biol. Chem. 286, 4380943815.
  • 28
    Schulz, C., Lytovchenko, O., Melin, J., Chacinska, A., Guiard, B., etal. ( 2011) Tim50′s presequence receptor domain is essential for signal driven transport across the TIM23 complex. J. Cell Biol. 195, 643656.
  • 29
    Qian, X., Gebert, M., Höpker, J., Yan, M., Li, J., etal. ( 2011) Structural basis for the function of Tim50 in the mitochondrial presequence translocase. J. Mol. Biol. 411, 513519.
  • 30
    Chacinska, A., Lind, M., Frazier, A. E., Dudek, J., Meisinger, C., etal. ( 2005) Mitochondrial presequence translocase: switching between TOM tethering and motor recruitment involves Tim21 and Tim17. Cell 120, 817829.
  • 31
    van der Laan, M., Meinecke, M., Dudek, J., Hutu, D.P., Lind, M., etal. ( 2007) Motor-free mitochondrial presequence translocase drives membrane integration of preproteins. Nat. Cell Biol. 9, 11521159.
  • 32
    Chacinska, A., van der Laan, M., Mehnert, C. S., Guiard, B., Mick, D. U., etal. ( 2010) Distinct forms of mitochondrial TOM-TIM supercomplexes define signal-dependent states of preprotein sorting. Mol. Cell. Biol. 30, 307318.
  • 33
    Gebert, M., Schrempp, S. G., Mehnert, C. S., Heißwolf, A. K., Oeljeklaus, S., etal. ( 2012). Mgr2 promotes coupling of the mitochondrial presequence translocase to partner complexes. J. Cell Biol. 197, 595604.
  • 34
    van der Laan, M., Wiedemann, N., Mick, D. U., Guiard, B., Rehling, P., etal. ( 2006) A role for Tim21 in membrane-potential-dependent preprotein sorting in mitochondria. Curr. Biol. 16, 22712276.
  • 35
    Rehling, P., Model, K., Brandner, K., Kovermann, P., Sickmann, A., etal. ( 2003) Protein insertion into the mitochondrial inner membrane by a twin-pore translocase. Science 299, 17471751.
  • 36
    Gebert, N., Gebert, M., Oeljeklaus, S., von der Malsburg, K., Stroud, D. A., etal. ( 2011). Dual function of Sdh3 in the respiratory chain and TIM22 protein translocase of the mitochondrial inner membrane. Mol. Cell 44, 811818.
  • 37
    Dukanovic, J. and Rapaport, D. ( 2011) Multiple pathways in the integration of proteins into the mitochondrial outer membrane. Biochim. Biophys. Acta 1808, 971980.
  • 38
    Paschen, S. A., Waizenegger, T., Stan, T., Preuss, M., Cyrklaff, M., etal. ( 2003) Evolutionary conservation of biogenesis of beta-barrel membrane proteins. Nature 426, 862866.
  • 39
    Wiedemann, N., Kozjak, V., Chacinska, A., Schönfisch, B., Rospert, S., etal. ( 2003) Machinery for protein sorting and assembly in the mitochondrial outer membrane. Nature 424, 565571.
  • 40
    Kutik, S., Stojanovski, D., Becker, L., Becker, T., Meinecke, M., etal. ( 2008) Dissecting membrane insertion of mitochondrial beta-barrel proteins. Cell 132, 10111024.
  • 41
    Meisinger, C., Rissler, M., Chacinska, A., Szklarz, L. K., Milenkovic, D., etal. ( 2004) The mitochondrial morphology protein Mdm10 functions in assembly of the preprotein translocase of the outer membrane. Dev. Cell 7, 6171.
  • 42
    Kornmann, B., Currie, E., Collins, S. R., Schuldiner, M., Nunnari, J., etal. ( 2009) An ER-mitochondria tethering complex revealed by a synthetic biology screen. Science 325, 477481.
  • 43
    Thornton, N., Stroud, D. A., Milenkovic, D., Guiard, B., Pfanner, N., etal. ( 2010) Two modular forms of the mitochondrial sorting and assembly machinery are involved in biogenesis of alpha-helical outer membrane proteins. J. Mol. Biol. 396, 540549.
  • 44
    Sideris, D. P. and Tokatlidis, K. ( 2010) Oxidative protein folding in the mitochondrial intermembrane space. Antioxid. Redox. Signal. 13, 11891204.
  • 45
    Stojanovski, D., Bragoszewski, P., and Chacinska, A. ( 2012) The MIA pathway: a tight bond between protein transport and oxidative folding in mitochondria. Biochim. Biophys. Acta 1823, 11421150.
  • 46
    Herrmann, J. M. and Riemer, J. ( 2012). Mitochondrial disulfide relay: redox-regulated protein import into the intermembrane space. J. Biol. Chem. 287, 44264433.
  • 47
    Chacinska, A., Pfannschmidt, S., Wiedemann, N., Kozjak, V., Sanjuán Szklarz, L. K., etal. ( 2004) Essential role of Mia40 in import and assembly of mitochondrial intermembrane space proteins. EMBO J. 23, 37353746.
  • 48
    Mesecke, N., Terziyska, N., Kozany, C., Baumann, F., Neupert, W., etal. ( 2005) A disulfide relay system in the intermembrane space of mitochondria that mediates protein import. Cell 121, 10591069.
  • 49
    Banci, L., Bertini, I., Cefaro, C., Ciofi-Baffoni, S., Gallo, A., etal. ( 2009) MIA40 is an oxidoreductase that catalyzes oxidative protein folding in mitochondria. Nat. Struct. Mol. Biol. 16, 198206.
  • 50
    Stojanovski, D., Milenkovic, D., Müller, J. M., Gabriel, K., Schulze-Specking,A., etal. ( 2008) Mitochondrial protein import: precursor oxidation in a ternary complex with disulfide carrier and sulfhydryl oxidase. J. Cell Biol. 183, 195202.
  • 51
    Bien, M., Longen, S., Wagener, N., Chwalla, I., Herrmann, J. M., etal. ( 2010) Mitochondrial disulfide bond formation is driven by intersubunit electron transfer in Erv1 and proofread by glutathione. Mol. Cell 37, 516528.
  • 52
    Böttinger, L., Gornicka, A., Czerwik, T., Bragoszewski, P., Loniewska-Lwowska, A., etal. ( 2012) In vivo evidence for cooperation of Mia40 and Erv1 in the oxidation of mitochondrial proteins. Mol. Biol. Cell 23, 39573969.
  • 53
    Kojer, K., Bien, M., Gangel, H., Morgan, B., Dick, T. P., etal. ( 2012) Glutathione redox potential in the mitochondrial intermembrane space is linked to the cytosol and impacts the Mia40 redox state. EMBO J. 31, 31693182.
  • 54
    Milenkovic, D., Ramming, T., Müller, J. M., Wenz, L. S., Gebert, N., etal. ( 2009) Identification of the signal directing Tim9 and Tim10 into the intermembrane space of mitochondria. Mol. Biol. Cell 20, 25302539.
  • 55
    Sideris, D. P., Petrakis, N., Katrakili, N., Mikropoulou, D., Gallo, A., etal. ( 2009) A novel intermembrane space-targeting signal docks cysteines onto Mia40 during mitochondrial oxidative folding. J. Cell Biol. 187, 10071022.
  • 56
    von der Malsburg, K., Müller, J. M., Bohnert, M., Oeljeklaus, S., Kwiatkowska, P., etal. ( 2011) Dual role of mitofilin in mitochondrial membrane organization and protein biogenesis. Dev. Cell 21, 694707.
  • 57
    Hoppins, S., Collins, S. R., Cassidy-Stone, A., Hummel, E., Devay, R. M., etal. ( 2011) A mitochondrial-focused genetic interaction map reveals a scaffold-like complex required for inner membrane organization in mitochondria. J. Cell Biol. 195, 323340.
  • 58
    Harner, M., Körner, C., Walther, D., Mokranjac, D., Kaesmacher, J., etal. ( 2011) The mitochondrial contact site complex, a determinant of mitochondrial architecture. EMBO J. 30, 43564370.
  • 59
    Alkhaja, A. K., Jans, D. C., Nikolov, M., Vukotic, M., Lytovchenko, O., etal. ( 2012) MINOS1 is a conserved component of mitofilin complexes and required for mitochondrial function and cristae organization. Mol. Biol. Cell 23, 247257.
  • 60
    Harner, M., Neupert, W., and Deponte, M. ( 2011) Lateral release of proteins from the TOM complex into the outer membrane of mitochondria. EMBO J. 30, 32323241.
  • 61
    Becker, T., Pfannschmidt, S., Guiard, B., Stojanovski, D., Milenkovic, D., etal. ( 2008) Biogenesis of the mitochondrial TOM complex: Mim1 promotes insertion and assembly of signal-anchored receptors. J. Biol. Chem. 283, 120127.
  • 62
    Hulett, J. M., Lueder, F., Chan, N. C., Perry, A. J., Wolynec, P., etal. ( 2008) The transmembrane segment of Tom20 is recognized by Mim1 for docking to the mitochondrial TOM complex. J. Mol. Biol. 376, 694704.
  • 63
    Popov-Celeketić, J., Waizenegger, T., and Rapaport, D. ( 2008) Mim1 functions in an oligomeric form to facilitate the integration of Tom20 into the mitochondrial outer membrane. J. Mol. Biol. 376, 671680.
  • 64
    Setoguchi, K., Otera, H., and Mihara, K. ( 2006) Cytosolic factor- and TOM-independent import of C-tail-anchored mitochondrial outer membrane proteins. EMBO J. 25, 56355647.
  • 65
    Kemper, C., Habib, S. J., Engl, G., Heckmeyer, P., Dimmer, K. S., etal. ( 2008) Integration of tail-anchored proteins into the mitochondrial outer membrane does not require any known import components. J. Cell Sci. 121, 19901998.
  • 66
    Krumpe, K., Frumkin, I., Herzig, Y., Rimon, N., Ozbalci, C., etal. ( 2012) Ergosterol content specifies targeting of tail-anchored proteins to mitochondrial outer membranes. Mol. Biol. Cell 23, 39273935.
  • 67
    Stojanovski, D., Guiard, B., Kozjak-Pavlovic, V., Pfanner, N., and Meisinger, C. ( 2007) Alternative function for the mitochondrial SAM complex in biogenesis of alpha-helical TOM proteins. J. Cell Biol. 179, 881893.
  • 68
    Hegde, R. S. and Keenan, R. J. ( 2011) Tail-anchored membrane protein insertion into the endoplasmic reticulum. Nat. Rev. Mol. Cell Biol. 12, 787798.
  • 69
    Diekert, K., Kispal, G., Guiard, B., and Lill, R. ( 1999) An internal targeting signal directing proteins into the mitochondrial intermembrane space. Proc. Natl. Acad. Sci. USA 96, 1175211757.
  • 70
    Potting, C., Wilmes, C., Engmann, T., Osman, C., and Langer, T. ( 2010) Regulation of mitochondrial phospholipids by Ups1/PRELI-like proteins depends on proteolysis and Mdm35. EMBO J. 29, 28882898.
  • 71
    Mick, D. U., Fox, T. D., and Rehling, P. ( 2011) Inventory control: cytochrome c oxidase assembly regulates mitochondrial translation. Nat. Rev. Mol. Cell Biol. 12, 1420.
  • 72
    Ott, M. and Herrmann, J. M. ( 2010) Co-translational membrane insertion of mitochondrially encoded proteins. Biochim. Biophys. Acta 1803, 767775.
  • 73
    Gruschke, S., Kehrein, K., Römpler, K., Gröne, K., Israel, L., etal. ( 2011) Cbp3-Cbp6 interacts with the yeast mitochondrial ribosomal tunnel exit and promotes cytochrome b synthesis and assembly. J. Cell. Biol. 193, 11011114.
  • 74
    Keil, M., Bareth, B., Woellhaf, M. W., Peleh, V., Prestele, M., etal. ( 2012) Oxa1-ribosome complexes coordinate the assembly of cytochrome C oxidase in mitochondria. J. Biol. Chem. 287, 3448434493.
  • 75
    Krüger, V., Deckers, M., Hildenbeutel, M., van der Laan, M., Hellmers, M., etal. ( 2012) The mitochondrial oxidase assembly protein1 (oxa1) insertase forms a membrane pore in lipid bilayers. J. Biol. Chem. 287, 3331433326.
  • 76
    Bohnert, M., Rehling, P., Guiard, B., Herrmann, J. M., Pfanner, N., etal. ( 2010) Cooperation of stop-transfer and conservative sorting mechanisms in mitochondrial protein transport. Curr. Biol. 20, 12271232.
  • 77
    Wagener, N., Ackermann, M., Funes, S., and Neupert, W. ( 2011) A pathway of protein translocation in mitochondria mediated by the AAA-ATPase Bcs1. Mol. Cell 44, 191202.
  • 78
    Mossmann, D., Meisinger, C., and Vögtle, F. N. ( 2012) Processing of mitochondrial presequences. Biochim. Biophys. Acta 1819, 10981106.
  • 79
    Anand, R., Langer, T., and Baker, M. J. Proteolytic control of mitochondrial function and morphogenesis. Biochim. Biophys. Acta, DOI:10.1016/j.bbamcr.2012.06.025.
  • 80
    Vögtle, F. N., Wortelkamp, S., Zahedi, R. P., Becker, D., Leidhold, C., etal. ( 2009) Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability. Cell 139, 428439.
  • 81
    Vögtle, F. N., Prinz, C., Kellermann, J., Lottspeich, F., Pfanner, N., etal. ( 2011) Mitochondrial protein turnover: role of the precursor intermediate peptidase Oct1 in protein stabilization. Mol. Biol. Cell 22, 21352143.
  • 82
    Varshavsky, A. The N-end rule pathway and regulation by proteolysis. Protein Sci., DOI:10.1002/pro.666.
  • 83
    Glick, B. S., Brandt, A., Cunningham, K., Müller, S., Hallberg, R. L., etal. ( 1992) Cytochromes c1 and b2 are sorted to the intermembrane space of yeast mitochondria by a stop-transfer mechanism. Cell 69, 809822.
  • 84
    Rugarli, E. I. and Langer, T. ( 2012) Mitochondrial quality control: a matter of life and death for neurons. EMBO J. 31, 13361349.
  • 85
    Nolden, M., Ehses, S., Koppen, M., Bernacchia, A., Rugarli, E. I., etal. ( 2005) The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria. Cell 123, 277289.
  • 86
    Bonn, F., Tatsuta, T., Petrungaro, C., Riemer, J., and Langer, T. ( 2011) Presequence-dependent folding ensures MrpL32 processing by the m-AAA protease in mitochondria. EMBO J. 30, 25452556.
  • 87
    Craig, E. A., Huang, P., Aron, R., and Andrew, A. ( 2006) The diverse roles of J-proteins, the obligate Hsp70 co-chaperone. Rev. Physiol. Biochem. Pharmacol. 156, 121.
  • 88
    Voos, W. Chaperone-protease networks in mitochondrial protein homeostasis. Biochim. Biophys. Acta, DOI:10.1016/j.bbamcr.2012.06.005.
  • 89
    Schilke, B., Williams, B., Knieszner, H., Pukszta, S., D'Silva, P., etal. ( 2006) Evolution of mitochondrial chaperones utilized in Fe-S cluster biogenesis. Curr. Biol. 16, 16601665.
  • 90
    Lill, R., Hoffmann, B., Molik, S., Pierik, A. J., Rietzschel, N., etal. ( 2012) The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism. Biochim. Biophys. Acta 1823, 14911508.
  • 91
    Weckbecker, D., Longen, S., Riemer, J., and Herrmann, J. M. (2012). Atp23 biogenesis reveals a chaperone-like folding activity of Mia40 in the IMS of mitochondria. EMBO J. 31:43484358.
  • 92
    Schreiner, B., Westerburg, H., Forné, I., Imhof, A., Neupert, W., etal. (2012). Role of the AAA protease Yme1 in folding of proteins in the intermembrane space of mitochondria. Mol. Biol. Cell 23:43354346.
  • 93
    Wittig, I. and Schägger, H. ( 2009). Supramolecular organization of ATP synthase and respiratory chain in mitochondrial membranes. Biochim. Biophys. Acta 1787, 672680.
  • 94
    Davies, K. M., Anselmi, C., Wittig, I., Faraldo-Gómez, J. D., and Kühlbrandt, W. ( 2012). Structure of the yeast F1Fo-ATP synthase dimer and its role in shaping the mitochondrial cristae. Proc. Natl. Acad. Sci. USA 109, 1360213607.
  • 95
    Frey, T. G. and Mannella, C.A. ( 2000). The internal structure of mitochondria. Trends Biochem. Sci. 25, 319324.
  • 96
    Frezza, C., Cipolat, S., Martins de Brito, O., Micaroni, M., Beznoussenko, G. V., etal. ( 2006). OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion. Cell 126, 177189.
  • 97
    Rabl, R., Soubannier, V., Scholz, R., Vogel, F., Mendl, N., etal. ( 2009) Formation of cristae and crista junctions in mitochondria depends on antagonism between Fcj1 and Su e/g. J. Cell Biol. 185, 10471063.
  • 98
    Wurm, C. A. and Jakobs, S. ( 2006) Differential protein distributions define two sub-compartments of the mitochondrial inner membrane in yeast. FEBS Lett. 580, 56285634.
  • 99
    Vogel, F., Bornhövd, C., Neupert, W., and Reichert, A. S. ( 2006) Dynamic subcompartmentalization of the mitochondrial inner membrane. J. Cell Biol. 175, 237247.
  • 100
    Stoldt, S., Wenzel, D., Hildenbeutel, M., Wurm, C. A., Herrmann, J. M., etal. ( 2012) The inner-mitochondrial distribution of Oxa1 depends on the growth conditions and on the availability of substrates. Mol. Biol. Cell 23, 22922301.
  • 101
    Zerbes, R. M., van der Klei, I. J., Veenhuis, M., Pfanner, N., van der Laan, M., etal. ( 2012) Mitofilin complexes: conserved organizers of mitochondrial membrane architecture. Biol. Chem. 393, 12471261.
  • 102
    Tatsuta, T. and Langer, T. ( 2008) Quality control of mitochondria: protection against neurodegeneration and ageing. EMBO J. 27, 306314.
  • 103
    Fischer, F., Hamann, A., and Osiewacz, H. D. ( 2012) Mitochondrial quality control: an integrated network of pathways. Trends Biochem. Sci. 37, 284292.
  • 104
    Baker, M. J., Mooga, V. P., Guiard, B., Langer, T., Ryan, M. T., etal. (2012). Impaired folding of the mitochondrial small TIM chaperones induces clearance by the i-AAA protease. J. Mol. Biol. 424:227239.
  • 105
    Livnat-Levanon, N. and Glickman, M. H. ( 2011) Ubiquitin-proteasome system and mitochondria—reciprocity. Biochim. Biophys. Acta 1809, 8087.
  • 106
    Escobar-Henriques, M., Westermann, B., and Langer, T. ( 2006) Regulation of mitochondrial fusion by the F-box protein Mdm30 involves proteasome-independent turnover of Fzo1. J. Cell Biol. 173, 645650.
  • 107
    Cohen, M. M., Leboucher, G. P., Livnat-Levanon, N., Glickman, M. H., and Weissman, A. M. ( 2008) Ubiquitin-proteasome-dependent degradation of a mitofusin, a critical regulator of mitochondrial fusion. Mol. Biol. Cell 19, 24572464.
  • 108
    Heo, J. M., Livnat-Levanon, N., Taylor, E. B., Jones, K. T., Dephoure, N., etal. ( 2010). A stress-responsive system for mitochondrial protein degradation. Mol. Cell 40, 465480.
  • 109
    Narendra, D. P., Jin, S. M., Tanaka, A., Suen, D. F., Gautier, C. A., etal. ( 2010) PINK1 is selectively stabilized on impaired mitochondria to activate Parkin. PLoS Biol. 8, e1000298.
  • 110
    Benard, G., Neutzner, A., Peng, G., Wang, C., Livak, F., etal. ( 2010) IBRDC2, an IBR-type E3 ubiquitin ligase, is a regulatory factor for Bax and apoptosis activation. EMBO J. 29, 14581471.
  • 111
    Moberg, P., Ståhl, A., Bhushan, S., Wright, S. J., Eriksson, A., etal. ( 2003) Characterization of a novel zinc metalloprotease involved in degrading targeting peptides in mitochondria and chloroplasts. Plant J. 36, 616628.
  • 112
    Glaser, E. and Alikhani, N. ( 2010) The organellar peptidasome, PreP: a journey from Arabidopsis to Alzheimer's disease. Biochim. Biophys. Acta 1797, 10761080.
  • 113
    Alikhani, N., Berglund, A. K., Engmann, T., Spånning, E., Vögtle, F. N., etal. ( 2011) Targeting capacity and conservation of PreP homologues localization in mitochondria of different species. J. Mol. Biol. 410, 400410.
  • 114
    Young, L., Leonhard, K., Tatsuta, T., Trowsdale, J., and Langer, T. ( 2001) Role of the ABC transporter Mdl1 in peptide export from mitochondria. Science 291, 21352138.
  • 115
    Haynes, C. M. and Ron, D. ( 2010) The mitochondrial UPR—protecting organelle protein homeostasis. J. Cell Sci. 123, 38493855.
  • 116
    Haynes, C. M., Yang, Y., Blais, S. P., Neubert, T. A., and Ron, D. ( 2010) The matrix peptide exporter HAF-1 signals a mitochondrial UPR by activating the transcription factor ZC376.7 in C. elegans. Mol. Cell 37, 529540.
  • 117
    Kanki, T., Wang, K., Cao, Y., Baba, M., and Klionsky, D. J. ( 2009) Atg32 is a mitochondrial protein that confers selectivity during mitophagy. Dev. Cell 17, 98109.
  • 118
    Okamoto, K., Kondo-Okamoto, N., and Ohsumi, Y. ( 2009) Mitochondria-anchored receptor Atg32 mediates degradation of mitochondria via selective autophagy. Dev. Cell 17, 8797.
  • 119
    Novak, I., Kirkin, V., McEwan, D. G., Zhang, J., Wild, P., etal. ( 2010) Nix is a selective autophagy receptor for mitochondrial clearance. EMBO Rep. 11, 4551.
  • 120
    Matsuda, N., Sato, S., Shiba, K., Okatsu, K., Saisho, K., etal. ( 2010) PINK1 stabilized by mitochondrial depolarization recruits Parkin to damaged mitochondria and activates latent Parkin for mitophagy. J. Cell Biol. 189, 211221.