Experts and Doctors on mitochondrial membrane transport proteins in Germany


Locale: Germany
Topic: mitochondrial membrane transport proteins

Top Publications

  1. Mokranjac D, Popov Celeketic D, Hell K, Neupert W. Role of Tim21 in mitochondrial translocation contact sites. J Biol Chem. 2005;280:23437-40 pubmed
    ..We propose that Tim21 binds to the trans site of the TOM complex thus keeping the two translocases in close contact. ..
  2. Eckert G, Renner K, Eckert S, Eckmann J, Hagl S, Abdel Kader R, et al. Mitochondrial dysfunction--a pharmacological target in Alzheimer's disease. Mol Neurobiol. 2012;46:136-50 pubmed publisher
    ..Thus, more studies are needed that focus on therapeutic strategies starting before severe disease progress. ..
  3. Kojer K, Bien M, Gangel H, Morgan B, Dick T, Riemer J. Glutathione redox potential in the mitochondrial intermembrane space is linked to the cytosol and impacts the Mia40 redox state. EMBO J. 2012;31:3169-82 pubmed publisher
    ..Taken together, we provide a comprehensive mechanistic picture of the IMS redox milieu and define the redox influences on Mia40 in living cells. ..
  4. Hönlinger A, Bömer U, Alconada A, Eckerskorn C, Lottspeich F, Dietmeier K, et al. Tom7 modulates the dynamics of the mitochondrial outer membrane translocase and plays a pathway-related role in protein import. EMBO J. 1996;15:2125-37 pubmed
    ..These results suggest that (i) Tom7 plays a role in sorting and accumulation of the preproteins at the outer membrane, and (ii) Tom7 and Tom6 perform complementary functions in modulating the dynamics of the outer membrane translocase. ..
  5. Osiewacz H. Regulation of the mitochondrial transition pore: impact on mammalian aging. Aging (Albany NY). 2011;3:10-1 pubmed
  6. Sirrenberg C, Endres M, FOLSCH H, Stuart R, Neupert W, Brunner M. Carrier protein import into mitochondria mediated by the intermembrane proteins Tim10/Mrs11 and Tim12/Mrs5. Nature. 1998;391:912-5 pubmed
    ..This interaction of Tim10 and Tim12 with the precursors depends on the presence of divalent metal ions. Both proteins contain a zinc-finger-like motif with four cysteines and bind equimolar amounts of zinc ions. ..
  7. Hill K, Model K, Ryan M, Dietmeier K, Martin F, Wagner R, et al. Tom40 forms the hydrophilic channel of the mitochondrial import pore for preproteins [see comment]. Nature. 1998;395:516-21 pubmed
    ..We conclude that Tom40 is the pore-forming subunit of the mitochondrial general import pore and that it constitutes a hydrophilic, approximately 22 A wide channel for the import of preproteins. ..
  8. Gellerich F, Gizatullina Z, Trumbeckaite S, Nguyen H, Pallas T, Arandarcikaite O, et al. The regulation of OXPHOS by extramitochondrial calcium. Biochim Biophys Acta. 2010;1797:1018-27 pubmed publisher
    ..The binding of these proteins to Ca2+-binding sites can impair the regulation by Ca2+, causing energetic depression and neurodegeneration. ..
  9. Vukotic M, Nolte H, König T, Saita S, Ananjew M, Kruger M, et al. Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria. Mol Cell. 2017;67:471-483.e7 pubmed publisher
    ..The dual function of AGK as lipid kinase and constituent of the TIM22 complex reveals that disturbances in both phospholipid metabolism and mitochondrial protein biogenesis contribute to the pathogenesis of Sengers syndrome. ..

More Information


  1. Becker L, Bannwarth M, Meisinger C, Hill K, Model K, Krimmer T, et al. Preprotein translocase of the outer mitochondrial membrane: reconstituted Tom40 forms a characteristic TOM pore. J Mol Biol. 2005;353:1011-20 pubmed publisher
  2. Milenkovic D, Kozjak V, Wiedemann N, Lohaus C, Meyer H, Guiard B, et al. Sam35 of the mitochondrial protein sorting and assembly machinery is a peripheral outer membrane protein essential for cell viability. J Biol Chem. 2004;279:22781-5 pubmed
    ..Sam35 is not required for the import of inner membrane or matrix targeted proteins. The presence of two essential proteins in the SAM complex, Sam35 and Sam50, indicates that it plays a central role in mitochondrial biogenesis. ..
  3. Sommer S, Leistner M, Aleksic I, Schimmer C, Alhussini K, Kanofsky P, et al. Impact of levosimendan and ischaemia-reperfusion injury on myocardial subsarcolemmal mitochondrial respiratory chain, mitochondrial membrane potential, Ca2+ cycling and ATP synthesis. Eur J Cardiothorac Surg. 2016;49:e54-62; discussion e62 pubmed publisher
    ..LS-dependent inhibition of ATP generation presumably resulted from complex IV and V limitations and lowered ΔΨm. However, a resulting impact of limited ATP synthesis on myocardial recovery remains arguable. ..
  4. Mirus O, Schleiff E. Prediction of beta-barrel membrane proteins by searching for restricted domains. BMC Bioinformatics. 2005;6:254 pubmed
    ..coli with high reliability. However, the reliability of the prediction is improved significantly by a combinatory approach of several programs. The consequences and usability of the developed scores are discussed. ..
  5. Mokranjac D, Berg A, Adam A, Neupert W, Hell K. Association of the Tim14.Tim16 subcomplex with the TIM23 translocase is crucial for function of the mitochondrial protein import motor. J Biol Chem. 2007;282:18037-45 pubmed
    ..Deletion of hydrophobic segments in both, Tim16 and Tim14, is lethal. We conclude that import into the matrix space of mitochondria requires association of the co-chaperones Tim16 and Tim14 with the TIM23 preprotein translocase. ..
  6. Tarasenko D, Barbot M, Jans D, Kroppen B, Sadowski B, Heim G, et al. The MICOS component Mic60 displays a conserved membrane-bending activity that is necessary for normal cristae morphology. J Cell Biol. 2017;216:889-899 pubmed publisher
    ..Our results show that membrane bending by Mic60 is an ancient mechanism, important for cristae formation, and had already evolved before α-proteobacteria developed into mitochondria. ..
  7. Reddehase S, Grumbt B, Neupert W, Hell K. The disulfide relay system of mitochondria is required for the biogenesis of mitochondrial Ccs1 and Sod1. J Mol Biol. 2009;385:331-8 pubmed publisher
    ..In conclusion, the disulfide relay system is crucial for the import of Ccs1, thereby affecting the transport of Sod1, and it can control the distribution of Ccs1 and Sod1 between the IMS of mitochondria and the cytosol. ..
  8. Sikor M, Mapa K, von Voithenberg L, Mokranjac D, Lamb D. Real-time observation of the conformational dynamics of mitochondrial Hsp70 by spFRET. EMBO J. 2013;32:1639-49 pubmed publisher
    ..These results indicate that the conformational cycle of Ssc1 is more elaborate than previously thought and provide insight into how the Hsp70s can perform a wide variety of functions. ..
  9. Bömer U, Meijer M, Maarse A, Hönlinger A, Dekker P, Pfanner N, et al. Multiple interactions of components mediating preprotein translocation across the inner mitochondrial membrane. EMBO J. 1997;16:2205-16 pubmed
    ..We conclude that the protein transport machinery of the mitochondrial inner membrane consists of dynamically interacting sub-complexes, each of which transiently binds mtHsp70. ..
  10. Schulz S, Schmitt S, Wimmer R, Aichler M, Eisenhofer S, Lichtmannegger J, et al. Progressive stages of mitochondrial destruction caused by cell toxic bile salts. Biochim Biophys Acta. 2013;1828:2121-33 pubmed publisher
    ..This suggests that ANT is one decisive target for toxic bile salts. To our knowledge this is the first report unraveling the consecutive steps leading to mitochondrial destruction by cell-toxic bile salts. ..
  11. 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
    ..In mitochondria as well as in vitro, Hot13 can be functionally replaced by zinc-binding chelators. We propose that Hot13 maintains Mia40 in a zinc-free state, thereby facilitating its efficient oxidation by Erv1. ..
  12. Brandner K, Mick D, Frazier A, Taylor R, Meisinger C, Rehling P. Taz1, an outer mitochondrial membrane protein, affects stability and assembly of inner membrane protein complexes: implications for Barth Syndrome. Mol Biol Cell. 2005;16:5202-14 pubmed
    ..We conclude that inactivation of Taz1 affects both assembly and stability of respiratory chain complexes in the inner membrane of mitochondria. ..
  13. Voos W, von Ahsen O, Muller H, Guiard B, Rassow J, Pfanner N. Differential requirement for the mitochondrial Hsp70-Tim44 complex in unfolding and translocation of preproteins. EMBO J. 1996;15:2668-77 pubmed
    ..This is consistent with a model that the dynamic interaction of mtHsp70 with Tim44 generates a pulling force on preproteins which supports unfolding during translocation. ..
  14. Becker T, Pfannschmidt S, Guiard B, Stojanovski D, Milenkovic D, Kutik S, et al. Biogenesis of the mitochondrial TOM complex: Mim1 promotes insertion and assembly of signal-anchored receptors. J Biol Chem. 2008;283:120-7 pubmed
    ..Thus, Mim1 plays an important role in the efficient assembly of the mitochondrial TOM complex. ..
  15. Stockburger C, Miano D, Baeumlisberger M, Pallas T, Arrey T, Karas M, et al. A Mitochondrial Role of SV2a Protein in Aging and Alzheimer's Disease: Studies with Levetiracetam. J Alzheimers Dis. 2016;50:201-15 pubmed publisher
    ..In conclusion, interfering with the SV2a protein at the mitochondrial level and thereby improving mitochondrial function might represent an additional therapeutic effect of levetiracetam to improve symptoms of late-onset AD. ..
  16. Stojanovski D, Guiard B, Kozjak Pavlovic V, Pfanner N, Meisinger C. Alternative function for the mitochondrial SAM complex in biogenesis of alpha-helical TOM proteins. J Cell Biol. 2007;179:881-93 pubmed
    ..Thus, the substrate specificity of SAM is not restricted to beta-barrel proteins but also includes the majority of alpha-helical Tom proteins. ..
  17. Terziyska N, Grumbt B, Bien M, Neupert W, Herrmann J, Hell K. The sulfhydryl oxidase Erv1 is a substrate of the Mia40-dependent protein translocation pathway. FEBS Lett. 2007;581:1098-102 pubmed
    ..Thus, Erv1 represents an unusual type of substrate of the Mia40-dependent import pathway. ..
  18. Merlin A, von Ahsen O, Craig E, Dietmeier K, Pfanner N. A mutant form of mitochondrial GrpE suppresses the sorting defect caused by an alteration in the presequence of cytochrome b2. J Mol Biol. 1997;273:1-6 pubmed
  19. Dietmeier K, Hönlinger A, Bömer U, Dekker P, Eckerskorn C, Lottspeich F, et al. Tom5 functionally links mitochondrial preprotein receptors to the general import pore. Nature. 1997;388:195-200 pubmed
    ..We conclude that Tom5 represents a functional link between surface receptors and GIP, and is part of an 'acid chain' that guides the stepwise transport of positively charged mitochondrial targeting sequences. ..
  20. Wiedemann N, Truscott K, Pfannschmidt S, Guiard B, Meisinger C, Pfanner N. Biogenesis of the protein import channel Tom40 of the mitochondrial outer membrane: intermembrane space components are involved in an early stage of the assembly pathway. J Biol Chem. 2004;279:18188-94 pubmed
    ..We propose that the biogenesis pathway of beta-barrel proteins of the outer mitochondrial membrane not only requires TOM and SAM components, but also involves components of the intermembrane space. ..
  21. Mokranjac D, Paschen S, Kozany C, Prokisch H, Hoppins S, Nargang F, et al. Tim50, a novel component of the TIM23 preprotein translocase of mitochondria. EMBO J. 2003;22:816-25 pubmed
    ..We suggest that Tim50 plays a crucial role in the transfer of preproteins from the TOM complex to the TIM23 complex through the intermembrane space. ..
  22. Frazier A, Dudek J, Guiard B, Voos W, Li Y, Lind M, et al. Pam16 has an essential role in the mitochondrial protein import motor. Nat Struct Mol Biol. 2004;11:226-33 pubmed
    ..Thus, Pam16 is a newly identified type of motor subunit and is required to promote a functional PAM reaction cycle, thereby driving preprotein import into the matrix. ..
  23. 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
    ..We suggest that this process reflects the evolutionary origin of the IMS from the periplasmic space of the prokaryotic ancestors of mitochondria. ..
  24. 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
    ..Moreover, Erv1 associated with Mia40 in a reductant-sensitive manner. We conclude that two essential proteins, Mia40 and Erv1, cooperate in the assembly pathway of small proteins of the mitochondrial IMS. ..
  25. Gross D, Burgard C, Reddehase S, Leitch J, Culotta V, Hell K. Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system. Mol Biol Cell. 2011;22:3758-67 pubmed publisher
    ..Thus the disulfide relay system is able to form, in addition to double disulfide bonds in twin Cx(n)C motifs, single structural disulfide bonds in complex protein domains. ..
  26. Siemen D, Ziemer M. What is the nature of the mitochondrial permeability transition pore and what is it not?. IUBMB Life. 2013;65:255-62 pubmed publisher
    ..Thereafter, this review will critically report about some of the unknown elements and hypotheses that had to be rejected. ..
  27. Dietmeier K, Zara V, Palmisano A, Palmieri F, Voos W, Schlossmann J, et al. Targeting and translocation of the phosphate carrier/p32 to the inner membrane of yeast mitochondria. J Biol Chem. 1993;268:25958-64 pubmed
    ..Yeast PiC/p32 is thus not only structurally homologous to the ADP/ATP carrier, but has a similar targeting mechanism and submitochondrial location, supporting its classification as a member of the inner membrane carrier family. ..
  28. Dekker P, Ryan M, Brix J, Muller H, Hönlinger A, Pfanner N. Preprotein translocase of the outer mitochondrial membrane: molecular dissection and assembly of the general import pore complex. Mol Cell Biol. 1998;18:6515-24 pubmed
    ..ii) Tom20 and Tom70 are not essential for the generation of the GIP complex. (iii) Tom6 functions as an assembly factor for Tom22, promoting its stable association with Tom40. ..
  29. Schneider H, Westermann B, Neupert W, Brunner M. The nucleotide exchange factor MGE exerts a key function in the ATP-dependent cycle of mt-Hsp70-Tim44 interaction driving mitochondrial protein import. EMBO J. 1996;15:5796-803 pubmed
    ..Subsequently, the release of mt-Hsp70 from the polypeptide chain is triggered by Mge1p which promotes release of ADP from mt-Hsp70. Rebinding of ATP to mt-Hsp70 completes the reaction cycle. ..
  30. Schlossmann J, Lill R, Neupert W, Court D. Tom71, a novel homologue of the mitochondrial preprotein receptor Tom70. J Biol Chem. 1996;271:17890-5 pubmed
    ..Thus, despite their strikingly similar biochemical properties, Tom71 and Tom70 do not perform identical functions. ..
  31. Voos W, Gambill B, Laloraya S, Ang D, Craig E, Pfanner N. Mitochondrial GrpE is present in a complex with hsp70 and preproteins in transit across membranes. Mol Cell Biol. 1994;14:6627-34 pubmed
    ..After being imported into the matrix, the preprotein could be coprecipitated only by antibodies against mt-hsp70. We propose that mt-hsp70 and MGE cooperate in membrane translocation of preproteins. ..
  32. Schmidt S, Strub A, Rottgers K, Zufall N, Voos W. The two mitochondrial heat shock proteins 70, Ssc1 and Ssq1, compete for the cochaperone Mge1. J Mol Biol. 2001;313:13-26 pubmed
    ..We conclude that mitochondria represent the unique case where two Hsp70s compete for the interaction with one nucleotide exchange factor. ..
  33. Hönlinger A, Kubrich M, Moczko M, Gartner F, Mallet L, Bussereau F, et al. The mitochondrial receptor complex: Mom22 is essential for cell viability and directly interacts with preproteins. Mol Cell Biol. 1995;15:3382-9 pubmed
    ..These results suggest that Mom22 plays an essential role in the mitochondrial receptor complex. It directly interacts with preproteins in transit and can perform receptor-like activities. ..
  34. Truscott K, Voos W, Frazier A, Lind M, Li Y, Geissler A, et al. A J-protein is an essential subunit of the presequence translocase-associated protein import motor of mitochondria. J Cell Biol. 2003;163:707-13 pubmed
    ..We conclude that the reaction cycle of the PAM of mitochondria involves an essential J-protein. ..
  35. Brandner K, Rehling P, Truscott K. The carboxyl-terminal third of the dicarboxylate carrier is crucial for productive association with the inner membrane twin-pore translocase. J Biol Chem. 2005;280:6215-21 pubmed
    ..We concluded that, in this case, a single structural repeat can drive inner membrane insertion, whereas all three related units contribute targeting information for outer membrane translocation. ..
  36. Dekker P, Martin F, Maarse A, Bömer U, Muller H, Guiard B, et al. The Tim core complex defines the number of mitochondrial translocation contact sites and can hold arrested preproteins in the absence of matrix Hsp70-Tim44. EMBO J. 1997;16:5408-19 pubmed
    ..We propose the hypothesis that mtHsp70 functions in protein import not only by direct interaction with preproteins, but also by exerting a regulatory effect on the Tim channel. ..
  37. Hofmann S, Rothbauer U, Mühlenbein N, Neupert W, Gerbitz K, Brunner M, et al. The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1.TIM13 complexes in the mitochondrial intermembrane space. J Biol Chem. 2002;277:23287-93 pubmed
    ..Thus, an assembly defect of DDP1 is the molecular basis of Mohr-Tranebjaerg syndrome in patients carrying the C66W mutation. ..
  38. Kovermann P, Truscott K, Guiard B, Rehling P, Sepuri N, Muller H, et al. Tim22, the essential core of the mitochondrial protein insertion complex, forms a voltage-activated and signal-gated channel. Mol Cell. 2002;9:363-73 pubmed
    ..Thus, a protein insertion complex can combine three essential functions, signal recognition, channel formation, and energy transduction, in one central component. ..
  39. Rothbauer U, Hofmann S, Mühlenbein N, Paschen S, Gerbitz K, Neupert W, et al. Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria. J Biol Chem. 2001;276:37327-34 pubmed
    ..Thus, the pathomechanism underlying the Mohr-Tranebjaerg syndrome may involve an impaired biogenesis of the human TIM23 complex causing severe pleiotropic mitochondrial dysfunction. ..
  40. Krimmer T, Rassow J, Kunau W, Voos W, Pfanner N. Mitochondrial protein import motor: the ATPase domain of matrix Hsp70 is crucial for binding to Tim44, while the peptide binding domain and the carboxy-terminal segment play a stimulatory role. Mol Cell Biol. 2000;20:5879-87 pubmed
    ..We conclude that the ATPase domain of mtHsp70 is essential for and directly interacts with Tim44, clearly separating the mtHsp70-Tim44 interaction from the mtHsp70-substrate interaction. ..
  41. Kurz M, Martin H, Rassow J, Pfanner N, Ryan M. Biogenesis of Tim proteins of the mitochondrial carrier import pathway: differential targeting mechanisms and crossing over with the main import pathway. Mol Biol Cell. 1999;10:2461-74 pubmed
  42. Wiedemann N, Kozjak V, Chacinska A, Schönfisch B, Rospert S, Ryan M, et al. Machinery for protein sorting and assembly in the mitochondrial outer membrane. Nature. 2003;424:565-71 pubmed
  43. van der Laan M, Meinecke M, Dudek J, Hutu D, Lind M, Perschil I, et al. Motor-free mitochondrial presequence translocase drives membrane integration of preproteins. Nat Cell Biol. 2007;9:1152-9 pubmed
    ..Thus, the minimal system for preprotein integration into the mitochondrial inner membrane is the presequence translocase, a cardiolipin-rich membrane and a membrane potential. ..
  44. Hörster F, Baumgartner M, Viardot C, Suormala T, Burgard P, Fowler B, et al. Long-term outcome in methylmalonic acidurias is influenced by the underlying defect (mut0, mut-, cblA, cblB). Pediatr Res. 2007;62:225-30 pubmed
    ..In addition, long-term outcome was dependent on the age cohort and cobalamin responsiveness. ..
  45. Milenkovic D, Gabriel K, Guiard B, Schulze Specking A, Pfanner N, Chacinska A. Biogenesis of the essential Tim9-Tim10 chaperone complex of mitochondria: site-specific recognition of cysteine residues by the intermembrane space receptor Mia40. J Biol Chem. 2007;282:22472-80 pubmed
    ..Mia40 selectively recognizes cysteine-containing IMS proteins in a site-specific manner in organello and in vitro. Our results indicate that Mia40 acts as a trans receptor in the biogenesis of mitochondrial IMS proteins. ..
  46. Rehling P, Model K, Brandner K, Kovermann P, Sickmann A, Meyer H, et al. Protein insertion into the mitochondrial inner membrane by a twin-pore translocase. Science. 2003;299:1747-51 pubmed
    ..Thus, protein insertion was driven by the coordinated action of a twin-pore complex in two voltage-dependent steps. ..
  47. Gabriel K, Milenkovic D, Chacinska A, Müller J, Guiard B, Pfanner N, et al. Novel mitochondrial intermembrane space proteins as substrates of the MIA import pathway. J Mol Biol. 2007;365:612-20 pubmed
    ..However, tagging of MIA substrates can interfere with their subcellular localization, indicating that the proper localization of mitochondrial IMS proteins requires the characterization of the authentic untagged proteins. ..
  48. Mick D, Dennerlein S, Wiese H, Reinhold R, Pacheu Grau D, Lorenzi I, et al. MITRAC links mitochondrial protein translocation to respiratory-chain assembly and translational regulation. Cell. 2012;151:1528-41 pubmed publisher
    ..We establish an unexpected molecular link between the TIM23 transport machinery and assembly of respiratory-chain complexes that regulate mitochondrial protein synthesis in response to their assembly state. ..
  49. Moro F, Sirrenberg C, Schneider H, Neupert W, Brunner M. The TIM17.23 preprotein translocase of mitochondria: composition and function in protein transport into the matrix. EMBO J. 1999;18:3667-75 pubmed
    ..Tim44 forms dimers which recruit two molecules of mt-Hsp70 to the sites of protein import. A sequential, hand-over-hand mode of interaction of these two mt-Hsp70.Tim44 complexes with a translocating polypeptide chain is proposed. ..
  50. Merlin A, Voos W, Maarse A, Meijer M, Pfanner N, Rassow J. The J-related segment of tim44 is essential for cell viability: a mutant Tim44 remains in the mitochondrial import site, but inefficiently recruits mtHsp70 and impairs protein translocation. J Cell Biol. 1999;145:961-72 pubmed
    ..The efficient cooperation of mtHsp70 with Tim44 facilitates the translocation of loosely folded preproteins and plays a crucial role in the import of preproteins which contain a tightly folded domain. ..
  51. Rassow J, Dekker P, van Wilpe S, Meijer M, Soll J. The preprotein translocase of the mitochondrial inner membrane: function and evolution. J Mol Biol. 1999;286:105-20 pubmed
  52. Albrecht R, Rehling P, Chacinska A, Brix J, Cadamuro S, Volkmer R, et al. The Tim21 binding domain connects the preprotein translocases of both mitochondrial membranes. EMBO Rep. 2006;7:1233-8 pubmed
    ..We also identified a core sequence of Tom22 that binds to Tim21. Furthermore, negatively charged amino-acid residues of Tom22 are important for binding to Tim21. Here we suggest a mechanism for the TOM-TIM interaction. ..
  53. Schubert A, Grimm S. Cyclophilin D, a component of the permeability transition-pore, is an apoptosis repressor. Cancer Res. 2004;64:85-93 pubmed
    ..Importantly, cyclophilin D is specifically up-regulated in human tumors of the breast, ovary, and uterus, suggesting that inhibition of the PT-pore via up-regulation of cyclophilin D plays a role in tumorigenesis. ..
  54. Meinecke M, Wagner R, Kovermann P, Guiard B, Mick D, Hutu D, et al. Tim50 maintains the permeability barrier of the mitochondrial inner membrane. Science. 2006;312:1523-6 pubmed
    ..Thus, the hydrophilic cis domain of Tim50 maintains the permeability barrier of mitochondria by closing the translocation pore in a presequence-regulated manner. ..
  55. Hofmann S, Rothbauer U, Mühlenbein N, Baiker K, Hell K, Bauer M. Functional and mutational characterization of human MIA40 acting during import into the mitochondrial intermembrane space. J Mol Biol. 2005;353:517-28 pubmed
    ..Taken together, we conclude that the biogenesis and function of MIA40 in the mitochondrial intermembrane space is dependent on redox processes involving conserved cysteine residues. ..
  56. Moczko M, Ehmann B, Gartner F, Hönlinger A, Schafer E, Pfanner N. Deletion of the receptor MOM19 strongly impairs import of cleavable preproteins into Saccharomyces cerevisiae mitochondria. J Biol Chem. 1994;269:9045-51 pubmed
    ..Interestingly, the primary sequence of MOM19 predicts the presence of a tetratricopeptide motif that was also found in MOM72, in the peroxisomal membrane protein PAS8/PAS10, and in several proteins involved in RNA synthesis or mitosis. ..
  57. van der Laan M, Chacinska A, Lind M, Perschil I, Sickmann A, Meyer H, et al. Pam17 is required for architecture and translocation activity of the mitochondrial protein import motor. Mol Cell Biol. 2005;25:7449-58 pubmed
    ..Our findings suggest that Pam17 is required for the correct organization of the Pam16-Pam18 complex and thus contributes to regulation of mtHsp70 activity at the inner membrane translocation site. ..
  58. Mokranjac D, Sichting M, Popov Celeketic D, Berg A, Hell K, Neupert W. The import motor of the yeast mitochondrial TIM23 preprotein translocase contains two different J proteins, Tim14 and Mdj2. J Biol Chem. 2005;280:31608-14 pubmed
    ..However, overexpressed Mdj2 fully restores the growth of cells lacking Tim14. We conclude that Mdj2 is a functional J protein and a component of the mitochondrial import motor. ..
  59. Geissler A, Rassow J, Pfanner N, Voos W. Mitochondrial import driving forces: enhanced trapping by matrix Hsp70 stimulates translocation and reduces the membrane potential dependence of loosely folded preproteins. Mol Cell Biol. 2001;21:7097-104 pubmed
  60. Chacinska A, Lind M, Frazier A, Dudek J, Meisinger C, Geissler A, et al. Mitochondrial presequence translocase: switching between TOM tethering and motor recruitment involves Tim21 and Tim17. Cell. 2005;120:817-29 pubmed
    ..Thus, the presequence translocase is not a static complex but switches between TOM tethering and PAM binding in a reaction cycle involving Tim21 and Tim17. ..
  61. Schmitt S, Ahting U, Eichacker L, Granvogl B, Go N, Nargang F, et al. Role of Tom5 in maintaining the structural stability of the TOM complex of mitochondria. J Biol Chem. 2005;280:14499-506 pubmed publisher
    ..We conclude that the main role of Tom5, rather than being a receptor, is maintaining the structural integrity of the TOM complex...
  62. Rassow J, Maarse A, Krainer E, Kubrich M, Muller H, Meijer M, et al. Mitochondrial protein import: biochemical and genetic evidence for interaction of matrix hsp70 and the inner membrane protein MIM44. J Cell Biol. 1994;127:1547-56 pubmed
    ..By binding to MIM44, mt-hsp70 is recruited at the protein import sites of the inner membrane, and preproteins arriving at MIM44 may be directly handed over to mt-hsp70. ..
  63. Truscott K, Kovermann P, Geissler A, Merlin A, Meijer M, Driessen A, et al. A presequence- and voltage-sensitive channel of the mitochondrial preprotein translocase formed by Tim23. Nat Struct Biol. 2001;8:1074-82 pubmed
    ..Thus, Tim23 forms a voltage-sensitive high-conductance channel with specificity for mitochondrial presequences. ..
  64. Geissler A, Krimmer T, Schönfisch B, Meijer M, Rassow J. Biogenesis of the yeast frataxin homolog Yfh1p. Tim44-dependent transfer to mtHsp70 facilitates folding of newly imported proteins in mitochondria. Eur J Biochem. 2000;267:3167-80 pubmed
    ..We conclude that Tim44 not only plays a role in protein translocation but also in the pathways of mitochondrial protein folding. ..
  65. Sirrenberg C, Bauer M, Guiard B, Neupert W, Brunner M. Import of carrier proteins into the mitochondrial inner membrane mediated by Tim22. Nature. 1996;384:582-5 pubmed
    ..Import of proteins of the AAC family is independent of Tim23, and import of matrix targeting signals containing preproteins is independent of Tim22. ..
  66. Schulz C, Lytovchenko O, Melin J, Chacinska A, Guiard B, Neumann P, et al. Tim50's presequence receptor domain is essential for signal driven transport across the TIM23 complex. J Cell Biol. 2011;195:643-56 pubmed publisher
    ..Our results establish Tim50 as the primary presequence receptor at the inner membrane and show that targeting signals and Tim50 regulate the Tim23 channel in an antagonistic manner. ..
  67. Förtsch J, Hummel E, Krist M, Westermann B. The myosin-related motor protein Myo2 is an essential mediator of bud-directed mitochondrial movement in yeast. J Cell Biol. 2011;194:473-88 pubmed publisher
    ..Accumulating genetic evidence suggests that maintenance of mitochondrial morphology, Ypt11, and retention of mitochondria in the bud contribute to Myo2-dependent inheritance of mitochondria. ..
  68. Adam A, Endres M, Sirrenberg C, Lottspeich F, Neupert W, Brunner M. Tim9, a new component of the TIM22.54 translocase in mitochondria. EMBO J. 1999;18:313-9 pubmed
    ..10.12 complex and mediates partial translocation of mitochondrial carriers proteins across the outer membrane. The TIM9.10.12 complex assists further translocation into the inner membrane in association with TIM22.54. ..
  69. Longen S, Bien M, Bihlmaier K, Kloeppel C, Kauff F, Hammermeister M, et al. Systematic analysis of the twin cx(9)c protein family. J Mol Biol. 2009;393:356-68 pubmed publisher
  70. Bauer M, Gempel K, Reichert A, Rappold G, Lichtner P, Gerbitz K, et al. Genetic and structural characterization of the human mitochondrial inner membrane translocase. J Mol Biol. 1999;289:69-82 pubmed
    ..Both TIM complexes display a native molecular mass of 110 kDa. We suggest that the structural organization of TIM23.17 preprotein translocases is conserved from low to high eukaryotes. ..
  71. 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
    ..Thus, oxidation driven by Mia40 and Erv1 determines vectorial transport of the precursors into the mitochondrial intermembrane space. ..
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    ..However, the NH(2)-terminal segment is required for Tom40 to become assembled into the TOM complex. A model for the biogenesis of Tom40 is presented. ..
  73. van der Laan M, Wiedemann N, Mick D, Guiard B, Rehling P, Pfanner N. A role for Tim21 in membrane-potential-dependent preprotein sorting in mitochondria. Curr Biol. 2006;16:2271-6 pubmed
    ..These findings suggest a new coupling mechanism in an energy-transducing membrane. ..
  74. Paschen S, Rothbauer U, Kaldi K, Bauer M, Neupert W, Brunner M. The role of the TIM8-13 complex in the import of Tim23 into mitochondria. EMBO J. 2000;19:6392-400 pubmed
    ..It is demonstrated that import of human Tim23 is dependent on a high membrane potential. A mechanism to explain the pathology of MTS is discussed. ..
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    ..We conclude that import of porin does not proceed through the action of Tom20 alone, but requires an intact outer membrane and involves at least four more subunits of the TOM machinery, including the general import pore. ..
  76. Rehling P, Wiedemann N, Pfanner N, Truscott K. The mitochondrial import machinery for preproteins. Crit Rev Biochem Mol Biol. 2001;36:291-336 pubmed
    ..Moreover, we are now approaching a new era in which elaborated techniques have already allowed and will enable us to gather information about the TOM and TIM complexes on an ultrastructural level. ..
  77. Alconada A, Kubrich M, Moczko M, Hönlinger A, Pfanner N. The mitochondrial receptor complex: the small subunit Mom8b/Isp6 supports association of receptors with the general insertion pore and transfer of preproteins. Mol Cell Biol. 1995;15:6196-205 pubmed
    ..These results suggest that Mom8b supports the cooperativity between receptors and the general insertion pore and facilitates the release of preproteins from import components and thereby promotes efficient transfer of preproteins. ..
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    ..We outline a novel mechanism in which chaperones are recruited for a specific targeting event by a membrane-bound receptor. ..