The mitochondrial machinery for import of precursor proteins

Methods in Molecular Biology

Volumn 390, Issue , 2007, Pages 99-117

  Gabriel, Kipros ^a   Pfanner, Nikolaus ^a  

^a NONE

Author keywords

Mitochondrial intermembrane space import and assembly; Mitochondrial protein import; Presequence translocase associated motor; Sorting and assembly machinery; Translocase of the inner mitochondrial membrane; Translocase of the outer mitochondrial membrane

Indexed keywords

CARRIER PROTEIN; PROTEIN PRECURSOR;

ARTICLE; BIOLOGICAL MODEL; ENZYMOLOGY; METABOLISM; MITOCHONDRION; PROTEIN TRANSPORT; RIBOSOME;

MEMBRANE TRANSPORT PROTEINS; MITOCHONDRIA; MITOCHONDRIAL MEMBRANE TRANSPORT PROTEINS; MODELS, BIOLOGICAL; PROTEIN PRECURSORS; PROTEIN TRANSPORT; RIBOSOMES;

FUNGI; NEUROSPORA CRASSA; SACCHAROMYCES CEREVISIAE;

EID: 36549083649     PISSN: 10643745     EISSN: None     Source Type: Book Series    
DOI: 10.1385/1-59745-466-4:99     Document Type: Article

References (101)

1. Green, D. R. (2005) Apoptotic pathways: ten minutes to dead. Cell 121, 671-674.
2. Beal, M. F. (2005) Mitochondria take center stage in aging and neurodegeneration. Ann. Neurol. 58, 495-505.
3. Neupert, W. (1997) Protein import into mitochondria. Annu Rev Biochem. 66, 863-917.
4. Dekker, P. J., Ryan, M. T., Brix, J., Müller, H., Hönlinger, A., and Pfanner, N. (1998) Preprotein translocase of the outer mitochondrial membrane: molecular dissection and assembly of the general import pore complex. Mol. Cell Biol. 18, 6515-6524.
5. Taylor, R. D. and Pfanner, N. (2004) The protein import and assembly machinery of the mitochondrial outer membrane. Biochim. Biophys. Acta 1658, 37-43.
6. Gabriel, K., Egan, B., and Lithgow, T. (2003) Tom40, the import channel of the mitochondrial outer membrane, plays an active role in sorting imported proteins. EMBO J. 11, 2380-2386.
7. Wiedemann, N., Kozjak, V., Chacinska, A., et al. (2003) Machinery for protein sorting and assembly in the mitochondrial outer membrane. Nature 424, 565-571.
8. Pfanner, N., Wiedemann, N., Meisinger, C., and Lithgow T. (2004) Assembling the mitochondrial outer membrane. Nat. Struct. Mol. Biol. 11, 1044-1048.
9. Wiedemann, N., Frazier, A. E., and Pfanner, N. (2004) The protein import machinery of mitochondria. J. Biol. Chem. 279, 14473-14476.
10. Jensen, R. E. and Dunn, C. D. (2002) Protein import into and across the mitochondrial inner membrane: role of the TIM23 and TIM22 translocons. Biochim. Biophys. Acta 1592, 25-34.
11. Chacinska, A., Rehling, P., Guiard, B., et al. (2003) Mitochondrial translocation contact sites: separation of dynamic and stabilizing elements in formation of a TOM-TIM-preprotein supercomplex. EMBO J. 22, 5370-5381.
12. Endo, T., Yamamoto, H., and Esaki, M. (2003) Functional cooperation and separation of translocators in protein import into mitochondria, the doublemembrane bounded organelles. J. Cell Sci. 116, 3259-3267.
13. Koehler, C. M. (2004) New developments in mitochondrial assembly. Annu Rev Cell Dev Biol. 20, 309-335.
14. Chacinska, A., Lind, M., Frazier, A. E., et al. (2005) Mitochondrial presequence translocase: switching between TOM tethering and motor recruitment involves Tim21 and Tim17. Cell 120, 817-829.
15. Oka, T. and Mihara, K. (2005) A railroad switch in mitochondrial protein import. Mol. Cell 18, 145-146.
16. Geissler, A., Rassow, J., Pfanner, N., and Voos, W. (2001) Mitochondrial import driving forces: enhanced trapping by matrix Hsp70 stimulates translocation and reduces the membrane potential dependence of loosely folded preproteins. Mol. Cell Biol. 21, 7097-7104.
17. Truscott, K. N., Voos, W., Frazier, A. E., et ai. (2003) A J-protein is an essential subunit of the presequence translocase-associated protein import motor of mitochondria. J. Cell Biol. 163, 707-713.
18. Rehling, P., Brandner, K., and Pfanner, N. (2004) Mitochondrial import and the twin-pore translocase. Nat. Rev. Mol. Cell Biol. 5, 519-530.
19. Chacinska, A., Pfannschmidt, S., Wiedemann, N., et al. (2004) Essential role of Mia40 in import and assembly of mitochondrial intermembrane space proteins. EMBO J. 23, 3735-3746.
20. Roise, D. and Schatz, G. (1988) Mitochondrial presequences. J. Biol. Chem. 263, 4509-4511.
21. von Heijne, G., Steppuhn, J., and Herrmann, R.G. (1989) Domain structure of mitochondrial and chloroplast targeting peptides. Eur. J. Biochem. 180, 535-545.
22. 1 presequence is a stop-transfer sequence specific for the mitochondrial inner membrane. EMBO J. 6, 2441-2448.
23. 1 promotes early divergence from the general mitochondrial import pathway and restricts the unfoldase activity of matrix Hsp70. EMBO J. 14, 6043-6057.
24. 2 branches from the general mitochondrial import pathway at the preprotein translocase of the inner membrane. J. Biol. Chem. 272, 30439-30446.
25. Herrmann, J. M. and Hell, K. (2005) Chopped, trapped or tacked-protein translocation into the IMS of mitochondria. Trends Biochem. Sci. 30, 205-211.
26. Brix, J., Rüdiger, S., Bukau, B., Schneider-Mergener, J., and Pfanner, N. (1999) Distribution of binding sequences for the mitochondrial import receptors Tom20, Tom22, and Tom70 in a presequence-carrying preprotein and a non-cleavable preprotein. J. Biol. Chem. 274, 16522-16530.
27. Egan, B., Beilharz, T., George, R., et al. (1999) Targeting of tail-anchored proteins to yeast mitochondria in vivo. FEBS Lett. 451, 243-248.
28. Beddoe, T. and Lithgow, T. (2002) Delivery of nascent polypeptides to the mitochondrial surface. Biochim Biophys Acta 1592, 35-39.
29. Marc, P., Margeot, A., Devaux, F., Blugeon, C., Corral-Debrinski, M., and Jacq, C. (2002) Genome-wide analysis of mRNAs targeted to yeast mitochondria. EMBO Rep. 3, 159-164.
30. Söllner, T., Griffiths, G., Pfaller, R., Pfanner, N., and Neupert, W. (1989) MOM19, an import receptor for mitochondrial precursor proteins. Cell 59, 1061-1070.
31. Söllner, T., Pfaller, R., Griffiths, G., Pfanner, N., and Neupert W. (1990) A mitochondrial import receptor for the ADP/ATP carrier. Cell 62, 107-115.
32. Hiñes, V., Brandt, A., Griffiths, G., Horstmann, H., Brütsch, H., and Schatz, G. (1990) Protein import into yeast mitochondria is accelerated by the outer membrane protein MAS70. EMBO J. 9, 3191-3200.
33. Lithgow, T., Junne, T., Suda, K., Gratzer, S., and Schatz G. (1994) The mitochondrial outer membrane protein Mas22p is essential for protein import and viability of yeast. Proc. Natl. Acad. Sci. USA 91, 11973-11977.
34. Abe, Y., Shodai, T., Muto, T., et al. (2000) Structural basis of presequence recognition by the mitochondrial protein import receptor Tom20. Cell 100, 551-560.
35. Bolliger, L., Junne, T., Schatz, G., and Lithgow, T. (1995) Acidic receptor domains on both sides of the outer membrane mediate translocation of precursor proteins into yeast mitochondria. EMBO J. 14, 6318-6326.
36. Hönlinger, A., Kübrich, M., Moczko, M., et al. (1995) The mitochondrial receptor complex: Mom22 is essential for cell viability and directly interacts with preproteins. Mol. Cell. Biol. 15, 3382-3389.
37. van Wilpe, S., Ryan, M. T., Hill, K., et al. (1999) Tom22 is a multifunctional organizer of the mitochondrial preprotein translocase. Nature 401, 485-489.
38. Model, K., Prinz, T., Ruiz, T., et al. (2002) Protein translocase of the outer mitochondrial membrane: role of import receptors in the structural organization of the TOM complex. J. Mol. Biol. 316, 657-666.
39. Keil, P., Weinzierl, A., Kiebler, M., Dietmeier, K., Söllner, T., and Pfanner, N. (1993) Biogenesis of the mitochondrial receptor complex. Two receptors are required for binding of MOM38 to the outer membrane surface. J. Biol. Chem. 268, 19177-19180.
40. Krimmer, T., Rapaport, D., Ryan, M. T., et al. (2001) Biogenesis of porin of the outer mitochondrial membrane involves an import pathway via receptors and the general import pore of the TOM complex. J. Cell. Biol. 152, 289-300.
41. 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, 41-50.
42. Börner. U., Pfanner, N. and Dietmeier, K. (1996) Identification of a third yeast mitochondrial Tom protein with tetratrico peptide repeats. FEBS Lett. 382, 153-158.
43. Schlossmann, J., Lill, R., Neupert, W., and Court, D. A. (1996) Tom71, a novel homologue of the mitochondrial preprotein receptor Tom70. J. Biol. Chem. 271, 17890-17895.
44. Baker, K. P., Schaniel, A., Vestweber, D., and Schatz, G. (1990) A yeast mitochondrial outer membrane protein essential for protein import and cell viability. Nature 348, 605-609.
45. Hill, K., Model, K., Ryan, M.,T., et al. (1998) Tom40 forms the hydrophilic channel of the mitochondrial import pore for preproteins. Nature 395, 516-521.
46. Model, K., Meisinger, C., Prinz, T., et al. (2001) MultiStep assembly of the protein import channel of the mitochondrial outer membrane. Nat. Struct. Biol. 8, 361-370.
47. Rapaport, D. (2005) How does the TOM complex mediate insertion of precursor proteins into the mitochondrial outer membrane? J. Cell. Biol. 171, 419-423.
48. Künkele, K.,P., Heins, S., Dembowski, M., et al. (1998) The preprotein translocation channel of the outer membrane of mitochondria. Cell 93, 1009-1019.
49. Schwartz, M. P. and Matouschek, A. (1999) The dimensions of the protein import channels in the outer and inner mitochondrial membranes. Proc. Natl. Acad. Sci. USA 96, 13086-13090.
50. Hoppins, S. C. and Nargang, F. E. (2004) The Tim8-Tim13 complex of Neurospora crassa functions in the assembly of proteins into both mitochondrial membranes. J. Biol. Chem. 279, 12396-12405.
51. Wiedemann, N., Truscott, K.,N., Pfannschmidt, S., Guiard, B., Meisinger, C., and Pfanner, N. (2004) 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. 279, 18188-18194.
52. Kozjak, V., Wiedemann, N., Milenkovic, D., et al. (2003) An essential role of Sam50 in the protein sorting and assembly machinery of the mitochondrial outer membrane. J. Biol. Chem. 278, 48520-8523.
53. Paschen, S. A., Waizenegger, T., Stan, T., et al. (2003) Evolutionary conservation of biogenesis of β-barrel membrane proteins. Nature 426, 862-866.
54. Gentle, I., Gabriel, K., Beech, P., Waller, R., and Lithgow, T. (2004) The Omp85 family of proteins is essential for outer membrane biogenesis in mitochondria and bacteria. J. Cell Biol. 164, 19-24.
55. Voulhoux, R., Bos, M. P., Geurtsen, J., Mois, M., and Tommassen, J. (2003) Role of a highly conserved bacterial protein in outer membrane protein assembly. Science 299, 262-265.
56. Milenkovic, D., Kozjak, V., Wiedemann, N., et al. (2004) Sam35 of the mitochondrial protein sorting and assembly machinery is a peripheral outer membrane protein essential for cell viability. J. Biol. Chem. 279, 22781-22785.
57. Waizenegger, T., Habib, S. J., Lech, M., et al. (2004) Tob38, a novel essential component in the biogenesis of β-barrel proteins of mitochondria. EMBO Rep. 5, 704-709.
58. Ishikawa, D., Yamamoto, H., Tamura, Y., Moritoh, K., and Endo, T. (2004) Two novel proteins in the mitochondrial outer membrane mediate β-barrel protein assembly. J. Cell Biol. 166, 621-627.
59. Meisinger, C., Rissler, M., Chacinska, A., et al. (2004) The mitochondrial morphology protein Mdm10 functions in assembly of the preprotein translocase of the outer membrane. Dev. Cell 1, 61-71.
60. Johnson, A. E. and van Waes, M. A. (1999) The translocon: a dynamic gateway at the ER membrane. Annu. Rev. Cell Dev. Biol. 15, 799-842.
61. Sadlish, H., Pitonzo, D., Johnson, A. E., and Skach, W. R. (2005) Sequential triage of transmembrane segments by Secóla during biogenesis of a native multispanning membrane protein. Nat. Struct. Mol. Biol. 12, 870-878.
62. Geissler, A., Chacinska, A., Truscott, K. N., et al. (2002) The mitochondrial presequence translocase: an essential role of Tim50 in directing preproteins to the import channel. Cell 111, 507-518.
63. Yamamoto, H., Esaki, M., Kanamori, T., Tamura, Y., Nishikawa, S., and Endo, T. (2002) Tim50 is a subunit of the TIM23 complex that links protein translocation across the outer and inner mitochondrial membranes. Cell 111, 519-528.
64. Truscott, K. N., Kovermann, P., Geissler, A., et al. (2001) A presequence- and voltage-sensitive channel of the mitochondrial preprotein translocase formed by Tim23. Nat. Struct. Biol. 8, 1074-1082.
65. 2 modulates the ΔΨ-dependence of translocation of the matrix-targeting sequence. Mol. Biol. Cell 11, 3977-3391.
66. Schneider, A., Behrens, M., Scherer, P., Pratje, E., Michaelis, G., and Schatz, G. (1991) Inner membrane protease I, an enzyme mediating intramitochondrial protein sorting in yeast. EMBO J. 10, 247-254.
67. Nunnari, J., Fox, T. D., and Walter, P. (1993) A mitochondrial protease with two catalytic subunits of nonoverlapping specificities. Science 262, 1997-2004.
68. Esser, K, Pratje, E., and Michaelis, G. (1996) SOM 1, a small new gene required for mitochondrial inner membrane peptidase function in Saccharomyces cerevisiae. Mol. Gen. Genet. 252, 437-445.
69. Arlt, H., Tauer, R., Feldmann, H., Neupert, W., and Langer, T. (1996) The YTA10-12 complex, an AAA protease with chaperone-like activity in the inner membrane of mitochondria. Cell 85, 875-885.
70. Esser, K., Tursun, B., Ingenhoven, M., Michaelis, G., and Pratje, E. (2002) A novel two-step mechanism for removal of a mitochondrial signal sequence involves the mAAA complex and the putative rhomboid protease Pcpl. J. Mol. Biol. 323, 835-843.
71. Schneider, H. C., Berthold, J., Bauer, M. F., et al. (1994) Mitochondrial Hsp70/MIM44 complex facilitates protein import. Nature 371, 768-774.
72. Rassow, J., Maarse, A. C., Krainer, E., et al. (1994) Mitochondrial protein import: biochemical and genetic evidence for interaction of matrix hsp70 and the inner membrane protein MIM44. J. Cell Biol, 127, 1547-1556.
73. Kronidou, N. G., Oppliger, W., Bolliger, L., et al. (1994) Dynamic interaction between Isp45 and mitochondrial Hsp70 in the protein import system of the yeast mitochondrial inner membrane. Proc. Natl. Acad. Sci. USA 91, 12818-12822.
74. Strub, A., Lim, J. H., Pfanner, N., and Voos, W. (2000) The mitochondrial protein import motor. Biol. Chem. 381, 943-949.
75. D'Silva, P. D., Schilke, B., Walter, W., Andrew, A., and Craig, E. A. (2003) J protein cochaperone of the mitochondrial inner membrane required for protein import into the mitochondrial matrix. Proc. Natl. Acad. Sci USA 100, 13839-13844.
76. Truscott, K. N., Voos, W., Frazier, A. E., et al. (2003) A J-protein is an essential subunit of the presequence translocase-associated protein import motor of mitochondria. J. Cell Biol. 163, 707-713
77. Mokranjac, D., Sichting, M., Neupert, W. and Hell, K. (2003) Tim14, a novel key component of the import motor of the TIM23 protein translocase of mitochondria. EMBO J. 22, 4945-4956.
78. Mokranjac, D., Sichting, M., Popov-Celeketic, D., Berg, A., Hell, K. and Neupert, W. (2005) The import motor of the yeast mitochondrial TIM23 preprotein translocase contains two different J proteins, Tim14 and Mdj2. J. Biol. Chem. 280, 31608-31614.
79. Li, Y., Dudek, J., Guiard, B., Pfanner, N., Rehling, P., and Voos, W. (2004) The presequence translocase-associated protein import motor of mitochondria. Paml6 functions in an antagonistic manner to Pam18. J. Biol. Chem. 279, 38047-38054.
80. van der Laan, M., Chacinska, A., Lind, M., et al. (2005) Pam17 is required for architecture and translocation activity of the mitochondrial protein import motor. Mol. Cell Biol. 25, 7449-7458.
81. Isaya, G., Miklos, D., and Rollins, R.A. (1994) MIP1, a new yeast gene homologous to the rat mitochondrial intermediate peptidase gene, is required for oxidative metabolism in Saccharomyces cerevisiae. Mol. Cell Biol. 14, 5603-5616.
82. Voos, W. and Röttgers, K. (2002) Molecular chaperones as essential mediators of mitochondrial biogenesis. Biochim. Biophys. Acta 1592, 51-62.
83. Pfanner, N. and Neupert, W. (1987) Distinct steps in the import of ADP/ATP carrier into mitochondria. J. Biol. Chem. 161, 7528-7536.
84. Truscott, K. N., Wiedemann, N., Rehling, P., et al. (2002) Mitochondrial import of the ADP/ATP carrier: the essential TIM complex of the intermembrane space is required for precursor release from the TOM complex. Mol. Cell Biol. 22, 7780-7789.
85. Sirrenberg, C., Endres, M., Fölsch, H., Stuart, R.A., Neupert, W., and Brunner, M. (1998) Carrier protein import into mitochondria mediated by the intermembrane proteins Tim10/Mrs11 and Timl2/Mrs5. Nature 391, 912-915.
86. Koehler, C. M., Merchant, S., Oppliger, W., et al. (1998) Tim9p, an essential partner subunit of Tim10p for the import of mitochondrial carrier proteins. EMBO J. 17, 6477-6486.
87. Adam, A., Endres, M., Sirrenberg, C., Lottspeich, F., Neupert, W., and Brunner, M. (1999) Tim9, a new component of the TIM22.54 translocase in mitochondria. EMBO J. 18, 313-319.
88. Paschen, S. A., Rothbauer, U., Kaldi, K., Bauer, M. F., Neupert, W., and Brunner, M. (2000) The role of the TIM8-13 complex in the import of Tim23 into mitochondria. EMBO J. 19, 6392-6400.
89. Curran S. P., Leuenberger, D., Oppliger, W. and Koehler, C. M. (2002) The Tim9p-Tim10p complex binds to the transmembrane domains of the ADP/ATP carrier. EMBO J. 21, 942-953.
90. Webb, C. T., Gormann, M. A., Lazarou, M., Ryan, M. T., and Gulbis, J. M. (2006) Crystal structure of the mitochondrial chaperone TIM910 reveals a six-bladed α-propeller. Mol. Cell 21, 123-133.
91. Wiedemann, N., Pfanner, N., and Chacinska, A. (2006) Chaperoning through the Mitochondrial Intermembrane space. Mol. Cell 21, 145-148.
92. Rehling, P., Brandner, K., and Pfanner, N. (2004) Mitochondrial import and the twin-pore translocase. Nat. Rev. Mol. Cell Biol. 5, 519-530.
93. Kerscher, 0., Holder, J., Srinivasan, M., Leung, R. S., and Jensen, R. E. (1997) The Tim54p-Tim22p complex mediates insertion of proteins into the mitochondrial inner membrane. J. Cell Biol. 139, 1663-1675.
94. Kovermann, P., Truscott, K. N., Guiard, B., et al. (2002) Tim22, the essential core of the mitochondrial protein insertion complex, forms a voltage-activated and signal-gated channel. Mol. Cell 9, 363-373.
95. Kerscher, O., Sepuri, N. B. and Jensen, R. E. (2000) Tim18p is a new component of the Tim54p-Tim22p translocon in the mitochondrial inner membrane. Mol. Biol. Cell 11, 103-116.
96. Naoé, M., Ohwa, Y., Ishikawa, D., et al. (2004) Identification of Tim40 that mediates protein sorting to the mitochondrial intermembrane space. J. Biol. Chem. 279,47815-47821.
97. Terziyska, N., Lutz, T., Kozany, C., et al. (2005) Mia40, a novel factor for protein import into the intermembrane space of mitochondria is able to bind metal ions. FEBS Lett. 579, 179-184.
98. Mesecke, N., Terziyska, N., Kozany, C., et al. (2005) A disulfide relay system in the intermembrane space of mitochondria that mediates protein import. Cell 121, 1059-1069.
99. Curran, S. P., Leuenberger, D., Leverich, E. P., Hwang, D. K., Beverly, K. N., and Koehler, C. M. (2004) The role of Hot13p and redox chemistry in the mitochondrial TIM22 import pathway. J. Biol. Chem. 279, 43744-43751.
100. Rissler, M., Wiedemann, N., Pfannschmidt, S., et al. (2005) The essential mitochondrial protein Ervl cooperates with Mia40 in biogenesis of intermembrane space proteins. J. Mol. Biol. 353, 485-492.
101. Allen, S., Balabanidou, V., Sideris, D. P., Lisowsky, T., and Tokatlidis, K. (2005) Ervl mediates the Mia40-dependent protein import pathway and provides a functional link to the respiratory chain by shuttling electrons to cytochrome c. J. Mol. Biol. 353, 937-944.