Mitochondria pp 235-266 | Cite as

Mitochondrial Biogenesis

  • Alexander Tzagoloff
Part of the Cellular Organelles book series (BLSC)


The in vivo mechanism of assembly of the mitochondrion poses formidable conceptual and experimental challenges to the investigator. The morphological complexity of the organelle coupled with the fact that the synthesis of the constituent proteins and lipids occurs in two spatially separate compartments of the cell indicates a highly ordered process whose most general features remain unknown.


Cytochrome Oxidase Mitochondrial Biogenesis Neurospora Crassa Cytochrome Oxidase Subunit Mitochondrial Protein Synthesis 
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Selected Readings

  1. Agsteribbe, E., Datema, R., and Kroon, A. M. (1974) Mitochondrial polysomes from Neurospora erassa, in The Biogenesis of Mitochondria ( A. M. Kroon and C. Saccone, eds.), Academic Press, New York, pp. 305–314.Google Scholar
  2. Attardi, G., and Ojala, D. (1971) Mitochondrial ribosomes in HeLa cells, Nature [New Bioi.] 229: 133.Google Scholar
  3. Barrell, B. G., Bankier, A. T., and Drouin, J. (1979) A different genetic code in human mitochondria, Nature 282: 189.PubMedCrossRefGoogle Scholar
  4. Barrell, B. G., Anderson, S., Bankier, A. T., de Bruijn, M. H. L., Chen, E., Coulson, A. R., Drouin, J., Eperon, I. C., Nierlich, D., Staden, R., and Young, I. G. (1980) Different pattern of codon recognition by mammalian mitochondrial tRNAs, Proc. Natl. Acad. Sci. U.S.A. 77: 3164.Google Scholar
  5. Beattie, D. S., Basford, R. E., and Koritz, S. B. (1967) The inner membrane as the site of the in vitro incorporation of L(14C)leucine into mitochondrial proteins, Biochemistry 6: 3099.PubMedCrossRefGoogle Scholar
  6. Blobel, G., and Dobberstein, B. (1975) Transfer of proteins across membranes I. Presence of proteolytically processed and unprocessed nascent immunoglobulin light chains on membrane- bound ribosomes of murine myeloma. II. Reconstitiution of functional rough microsomes from heterologous components, J. Cell Biol. 67: 835, 852.PubMedCrossRefGoogle Scholar
  7. Blok, M. C., Wirtz, K. W. A., and Scherphof, G. L. (1969) Exchange of phospholipids between microsomes and inner and outer membranes of rat liver, Biochim. Biophys. Acta 233: 61.Google Scholar
  8. Bonitz, S. G., Berlani, R., Coruzzi, G., Li, M., Macino, G., Nobrega, F. G., Nobrega, M. P., Thalenfeld, B., and Tzagoloff, A. (1980) Codon recognition rules in yeast mitochondria, Proc. Natl. Acad. Sci. U.S.A. 77: 3167.PubMedCrossRefGoogle Scholar
  9. Borst, P. (1972) Mitochondrial nucleic acids, Annu. Rev. Biochem. 41: 333.PubMedCrossRefGoogle Scholar
  10. Chua, N.-H., and Schmidt, G. W. (1979) Transport of proteins into mitochondria and chloroplasts, J. Cell Biol. 81: 461.PubMedCrossRefGoogle Scholar
  11. Conboy, J. G., Kalousek, F., and Rosenberg, L. E. (1979) In vitro synthesis of a putative precursor of mitochondrial ornithine transcarbamylase, Proc. Natl. Acad. Sci. U.S.A. 76: 5724.PubMedCrossRefGoogle Scholar
  12. Cote, C., Solioz, M., and Schatz, G. (1979) Biogenesis of the cytochrome bct complex of yeast mitochondria. A precursor form of the cytoplasmically made subunit V, J. Biol. Chem. 254: 1437.PubMedGoogle Scholar
  13. Davidson, J. B., and Stanacev, N. Z. (1971) Biosynthesis of cardiolipin in mitochondria isolated from guinea pig liver, Biochem. Biophys. Res. Commun. 6: 1191.CrossRefGoogle Scholar
  14. Grivell, L. A., Reijnders, L., and Borst, P. (1971) The effect of temperature and ionic strength on the electrophoretic mobility of yeast mitochondrial RNA, Eur. J. Biochem. 19: 64.PubMedCrossRefGoogle Scholar
  15. Hallermayer, G., Zimmermann, R., and Neupert, W. (1977) Kinetic studies of the transport of cytoplasmically synthesized proteins into mitochondria of intact cells of N. crassa, Eur. J. Biochem. 81: 523.CrossRefGoogle Scholar
  16. Hare, J. F., Ching, E., and Attardi, G. (1980) Isolation, subunit composition and site of synthesis of human cytochrome c oxidase, Biochemistry 19: 2023PubMedCrossRefGoogle Scholar
  17. Heckman, J. E., Hecker, L. I., Schwartzbach, S. D., Barnett, W. E., Baumstark, B., and RajBhandary, U. L. (1978) Structure and function of initiator tRNA from the mitochondria of Neurospora crassa, Cell 13: 83.Google Scholar
  18. Heckman, J. E., Sarnoff, J., Alzner-Deweerd, B., Yin, S., and RajBhandary, U. L. (1980) Novel features in the genetic code and codon reading patterns in Neurospora crassa mitochondria based on sequences of six mitochondrial tRNAs, Proc. Natl. Acad. Sci. U.S.A. 77: 3159.PubMedCrossRefGoogle Scholar
  19. Koch, G. (1976) Synthesis of the mitochondrial inner membrane in cultured Xenopus laevis oocytes, J. Biol. Chem. 251: 6097.PubMedGoogle Scholar
  20. Kovac, L. (1974) Biochemical mutants: An approach to mitochondrial energy coupling, Biochim. Biophys. Acta 346: 101.PubMedGoogle Scholar
  21. Lamb, A. J., Clark-Walker, G. D., and Linnane, A. W. (1968) The biogenesis of mitochondria. The differentiation of mitochondrial and cytoplasmic protein synthesizing systems in vitro by antibiotics, Biochem. Biophys. Acta 161: 415.PubMedGoogle Scholar
  22. Lambowitz, A. M., Ghua, N.-H, and Luck, D. J. L. (1976) Mitochondrial ribosome assembly in Neurospora. Preparation of mitochondrial ribosomal precursor particles, sites of synthesis of mitochondrial ribosomal proteins and studies on the poky mutant, J. Mol. Biol. 107: 223.PubMedCrossRefGoogle Scholar
  23. Leaver, D. J., and Harmey, M. A. (1972) Isolation and characterization of mitochondrial ribosomes from higher plants, Biochem. J. 129: 37 p.Google Scholar
  24. Lewin, A. S., Gregor, I., Mason, T. L., Nelson, N., and Schatz, G. (1980) Cytoplasmically made subunits of yeast mitochondrial F1-ATPase and cytochrome c oxidase are synthesized as individual precursors, not as polyproteins, Proc. Natl. Acad. Sci. U.S.A. 77: 3998.PubMedCrossRefGoogle Scholar
  25. Lizardi, P. M., and Luck, D. J. L. (1972) The intracellular site of synthesis of mitochondrial ribosomal proteins in N. crassa, J. Cell. Biol. 54: 56.CrossRefGoogle Scholar
  26. Luck, D. J. L. (1963) Formation of mitochondria in Neurospora crassa. A quantitative radioautographic study, J. Cell Biol. 16: 483.PubMedCrossRefGoogle Scholar
  27. Luck, D. J. L. (1965) Formation of mitochondria in Neurospora crassa. A study based on mitochondrial density changes, J. Cell Biol. 24: 461.PubMedCrossRefGoogle Scholar
  28. Maccecchini, M.-L., Rudin, Y., Blobel, B., and Schatz, G. (1979) Import of proteins into mitochondria. Precursor forms of the extramitochondrially made F1subunits in yeast, Proc. Natl. Acad. Sci. U.S.A. 76: 343.PubMedCrossRefGoogle Scholar
  29. Martin, N. C., Rabinowitz, M., and Fukuhara, H. (1977) Yeast mitochondrial DNA specific tRNAs for 19 amino acids. Deletion mapping of the tRNA genes, Biochemistry 21: 4672.Google Scholar
  30. Mason, T. L., and Schatz, G. (1973) Cytochrome oxidase of Baker’s yeast II. Site of translation of the protein components, J. Biol. Chem. 248: 1355.PubMedGoogle Scholar
  31. McLean, J. R., Cohn, G. L., Brandt, I. K., and Simpson, M. V. (1958) Incorporation of labeled amino acids into the protein of muscle and liver mitochondria, J. Biol. Chem. 223: 657.Google Scholar
  32. Mihara, K., and Blobel, G. (1980) The four cytoplasmically made subunits of yeast mitochondrial cytochrome c oxidase are synthesized individually and not as a polyprotein, Proc. Natl. Acad. Sci. U.S.A. 77: 4160.PubMedCrossRefGoogle Scholar
  33. Neher, E.-M., Harmey, M. A., Hennig, B., Zimmermann, R., and Neupert, W. (1980) Post-translational transport of proteins in the assembly of mitochondrial membranes, in The Organization and Expression of the Mitochondrial Genome ( A. M. Kroon and C. Saccone, eds.), North-Holland, Amsterdam, pp. 413–422.Google Scholar
  34. Noller, H. F., and Woese, C. R. (1981) Secondary structure of 16 S ribosomal RNA, Science 212: 403.PubMedCrossRefGoogle Scholar
  35. O’Brien, T. W. (1971) The general occurrence of 55 S ribosomes in mammalian liver mitochondria, J. Biol. Chem. 246: 3409.PubMedGoogle Scholar
  36. Plattner, H., Salpeter, M. M., Saltzgaber, J., and Schatz, G. (1970) Promitochondria of anaerobically grown yeast IV. Conversion into respiring mitochondria, Proc. Natl. Acad. Sci. U.S.A. 66: 1252.PubMedCrossRefGoogle Scholar
  37. Pring, D. R. (1974) Maize mitochondria. Purification and characterization of ribosomes and ribosomal ribonucleic acid, Plant Physiol. 53: 677.PubMedCrossRefGoogle Scholar
  38. Raymond, Y., and Shore, G. C. (1979) The precursor for carbamyl phosphate synthetase is transported to mitochondria via a cytosolic route, J. Biol. Chem. 254: 9335.PubMedGoogle Scholar
  39. Robberson, D., Aloni, Y., and Attardi, G. (1971) Expression of the mitochondrial genome in HeLa cells. VI. Size determination of mitochondrial ribosomal RNA by electron microscopy, J. Mol. Biol. 60: 473.PubMedCrossRefGoogle Scholar
  40. Roodyn, D. B., Reix, P. J., and Work, T. S. (1961) Protein synthesis in mitochondria. Requirements for the incorporation of radioactive amino acids into mitochondrial protein, Biochem. J. 80: 9.PubMedGoogle Scholar
  41. Sager, R. (1972) Cytoplasmic Genes and Organelles, Academic Press, New York.Google Scholar
  42. Schatz, G. (1968) Impaired binding of mitochondrial adenosine triphosphatase in the cytoplasmic ‘petite’ mutant of Saccharomyces cerevisiae, J. Biol. Chem. 243: 2192.PubMedGoogle Scholar
  43. Schatz, G., and Mason, T. L. (1974) The biosynthesis of mitochondrial proteins, Annu. Rev. Biochem. 43: 51.CrossRefGoogle Scholar
  44. Schatz, G., Groot, G. S. P., Mason, T. L., Rouslin, W., Wharton, D. C., and Saltzgaber, J. (1972) Biogenesis of mitochondrial inner membrane in baker’s yeast, Fed. Proc. 31: 21.Google Scholar
  45. Schwab, A. J., Sebald, W., and Weiss, H. (1972) Different pool sizes of the precursor polypeptides of cytochrome oxidase from Neurospora crassa, Eur. J. Biochem. 30: 511.PubMedCrossRefGoogle Scholar
  46. Sebald, W. (1977) Biogenesis of mitochondrial ATPase, Biochim. Biophys. Acta 463: 1.PubMedGoogle Scholar
  47. Sebald, W., Hoppe, J., and Wächter, E. (1979) Amino acid sequence of the ATPase proteolipid from mitochondria, chloroplasts and bacteria, in Function and Molecular Aspects of Biomembrane Transport ( E. Quagliariello, F. Palmieri, S. Papa and M. Klingenberg, eds.), North-Holland, Amsterdam, pp. 63–74.Google Scholar
  48. Sevarino, K. A., and Poyton, R. O. (1980) Mitochondrial membrane biogenesis. Identification of a precursor to yeast cytochrome c oxidase subunit II, an integral polypeptide, Proc. Natl. Acad. Sci. U.S.A. 77: 142.PubMedCrossRefGoogle Scholar
  49. Sherman, F., and Slonimski, P. P. (1964) Respiratory deficient mutants in yeast II. Biochemistry, Biochim. Biophys. Acta 90: 1.PubMedCrossRefGoogle Scholar
  50. Sherman, F., and Stewart, J. W. (1971) Genetics and biosynthesis of cytochrome c, Annu. Rev. Genet. 5: 257.PubMedCrossRefGoogle Scholar
  51. Shine, J., and Dalgarno, L. (1974) The 3’-terminal sequence of Escherichia coli 16 S ribosomal RNA: Complementarity to nonsense triplets and ribosome binding sites, Proc. Natl. Acad. Sci. U.S.A. 71: 1342.PubMedCrossRefGoogle Scholar
  52. Shore, G. C., Carignan, P., and Raymond, Y. (1979) In vitro synthesis of a putative precursor to the mitochondrial enzyme, carbamyl phosphate synthetase, J. Biol. Chem. 254: 3141.PubMedGoogle Scholar
  53. Slonimski, P. P. (1953) La Formation des Enzymes Respiratoire chez la Levure, Masson, Paris.Google Scholar
  54. Tzagoloff, A. (1969) Assembly of the mitochondrial membrane system II. Synthesis of the mitochondrial adenosine triphosphatase F1, J. Biol. Chem. 244: 5027.PubMedGoogle Scholar
  55. Tzagoloff, A., Akai, A., and Needleman, R. (1975) Assembly of the mitochondrial membrane system. Isolation of nuclear mutants of S. cerevisiae with specific defects in mitochondrial functions, J. Biol. Chem. 250: 8228.PubMedGoogle Scholar
  56. Tzagoloff, A., Macino, G., and Sebald, W. (1979) Mitochondrial genes and translation products, Annu. Rev. Biochem. 48: 419.PubMedCrossRefGoogle Scholar
  57. Utter, M. F., Duell, E. A., and Bernofsky, C. (1968) Alterations in the respiratory enzymes of the mitochondria of growing and resting yeast, in Aspects of Yeast Metabolism ( A. K. Mills, ed.) Blackwell, Oxford, pp. 197–212.Google Scholar
  58. Weiss, H., and Ziganke, B. (1974) Cytochrome b in Neurospora crassa mitochondria. Site of translation of the heme protein, Eur. J. Biochem. 41: 63.PubMedCrossRefGoogle Scholar
  59. Wickner, W. (1979) The assembly of proteins into biological membranes. The membrane trigger hypothesis, Annu. Rev. Biochem. 48: 23.PubMedCrossRefGoogle Scholar
  60. Wirtz, K. W. A., and van Deenen, L. L. M. (1977) Phospholipid-exchange proteins: A new class of intracellular lipoproteins, Trends Biochem. Sci. 2: 49.CrossRefGoogle Scholar
  61. Wirtz, K. W. A., Kamp, H. H., and van Deenen, L. L. M. (1972) Isolation of a protein from beef liver which specifically stimulates the exchange of phosphatidylcholine, Biochim. Biophys. Acta 274: 607.Google Scholar
  62. Zimmermann, R., Paluch, U., and Neupert, W. (1979) Cell-free synthesis of cytochrome c, FEBS Lett. 108: 141.PubMedCrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1982

Authors and Affiliations

  • Alexander Tzagoloff
    • 1
  1. 1.Columbia UniversityNew YorkUSA

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