Skip to main content
SpringerLink
Log in

Microcompartmentation of energy metabolism at the outer mitochondrial membrane: Role in diabetes mellitus and other diseases

  • Published:
Journal of Bioenergetics and Biomembranes Aims and scope Submit manuscript

Abstract

Complexes made up of the kinases, hexokinase and glycerol kinase, together with the outer mitochondrial membrane voltage-dependent anion channel (VDAC) protein, porin, and the inner mitochondrial membrane protein, the adenine nucleotide translocator, are involved in tumorigenesis, diabetes mellitus, and central nervous system function. Identification of these two mitochondrial membrane proteins, along with an 18 kD protein, as components of the peripheral benzodiazepine receptor, provides independent confirmation of the interaction of porin and the adenine nucleotide translocator to form functional contact sites between the inner and outer mitochondrial membranes. We suggest that these are dynamic structures, with channel conductances altered by the presence of ATP, and that ligand-mediated conformational changes in the porin-adenine nucleotide translocator complexes may be a general mechanism in signal transduction.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Adams, V., and McCabe, E. R. B. (1994). InProceedings of NATO ARW, Molecular Biology of Mitochondrial Transport Systems (Colombini, M., and Fore, M., eds.), Springer-Verlag, Berlin, in press.

    Google Scholar 

  • Adams, V., Griffin, L., Towbin, J., Gelb, B., Worley, K., and McCabe, E. R. B. (1991a).Biochem. Med. Metab. Biol. 45 271–291.

    Google Scholar 

  • Adams, V., Griffin, L. D., Gelb, B. D., and McCabe, E. R. B. (1991b).Biochem. Biophys. Res. Commun. 177 1101–1106.

    Google Scholar 

  • Andreone, T., Printz, R. L., Pilkis, S. J., Magnuson, M. A., and Granner, D. K. (1989).J. Biol. Chem. 264 363–369.

    Google Scholar 

  • Arora, K. K., and Pedersen, P. L. (1988).J. Biol. Chem. 263 17422–17428.

    Google Scholar 

  • Arora, K. K., and Pedersen, P. L. (1993).Arch. Biochem. Biophys. 304 515–518.

    Google Scholar 

  • Ballinger, S. W., Shoffner, J. M., Hedaya, E. V., Trounce, I., Polak, M. A., Koontz, D. A., and Wallace, D. C. (1992).Nature Genet. 1 11–15.

    Google Scholar 

  • Barinaga, M. (1990).Science 250 506–507.

    Google Scholar 

  • Bessman, S. P. (1972).Isr. J. Med. Sci. 8 344–351.

    Google Scholar 

  • Bessman, S. P., Borrebaek, B., Geiger, P. J., and Ben-Or, S. (1978). InMicroenvironments and Metabolic Compartmentation (Srere, P. A., and Estabrook, R. W., eds.), Academic Press, New York, pp. 111–128.

    Google Scholar 

  • Blachly-Dyson, E., Zambronicz, E. B., Yu, W. H., Adams, V., McCabe, E. R. B., Adelman, J., Colombini, M., and Forte, M. (1993).J. Biol. Chem. 268 1835–1841.

    Google Scholar 

  • Blachly-Dyson, E., Baldini, A., Litt, M., McCabe, E. R. B., and Forte, M. (1994).Genomics, in press.

  • Borrebaek, B., and Spydevold, O. (1969).Diabetologia 5 42–43.

    Google Scholar 

  • Brdiczka, D., and Wicker, U. (1994). InProceedings of NATO ARW, Molecular Biology of Mitochondrial Transport Systems (Colombini, M., and Forte, M., eds.), Springer-Verlag, Berlin, in press.

    Google Scholar 

  • Bu, X., and Rotter, J. I. (1993).Lancet 342 598–600.

    Google Scholar 

  • Bustamante, E., Morris, H. P., and Pedersen, P. L. (1981).J. Biol. Chem. 256 8699–8704.

    Google Scholar 

  • Denis-Pouxviel, C., Riesinger, I., Buehler, C., Brdiczka, D., and Murat, J. C. (1987).Biochim. Biophys. Acta 902 335–348.

    Google Scholar 

  • Epstein, P., Boschero, A., Atwater, I., Cai, X., and Overbeek, P. (1993).Proc. Natl. Acad. Sci. USA 89 12038–12042.

    Google Scholar 

  • Fieck, C., Benz, R., Roos, N., and Brdiczka, D. (1982).Biochim. Biophys. Acta 688 429–440.

    Google Scholar 

  • Froguel, P., Vaxillaire, M., Sun, F., Velho, G., Zouali, H., Butel, M. O., Lesage, S., Vionnet, N., Clément, K., Fougerousse, F., Tanizawa, Y., Weissenbach, J., Beckmann, J. S., Lathrop, G. M., Pass, P., Permutt, M. A., and Cohen, D. (1992).Nature (London) 356 162–164.

    Google Scholar 

  • Froguel, P., Zouali, H., Vionnet, N., Velho, G., Vaxillaire, M., Sun, F., Lesage, S., Stoffel, M., Takeda, J., Passa, P., Permutt, A., Beckmann, J., Bell, G., and Cohen, D. (1993).N. Engl. J. Med. 328 697–702.

    Google Scholar 

  • Gelb, B. D., Adams, V., Jones, S. N., Griffin, L. D., MacGregor, G. R., and McCabe, E. R. B. (1992).Proc. Natl. Acad. Sci. USA 89 202–206.

    Google Scholar 

  • Gellerich, F. N., Wagner, M., Kapischke, M., Wicker, U., and Bridiczka, D. (1993).Biochim. Biophys. Acta 1142 217–227.

    Google Scholar 

  • Gerbitz, K. D., Paprotta, A., Jaksch, M., Zierz, S., and Drechsel, J. (1993).FEBS Lett. 321 194–196.

    Google Scholar 

  • Gidh-Jain, M., Takeda, J., Xu, L. Z., Lange, A. J., Vionnet, N., Stoffel, M., Froguel, P., Velho, G., Sun, F., Cohen, D., Patel, P., Lo, Y.-M. D., Hattersley, A. T., Luthman, H., Wedell, A., St. Charles, R., Harrison, R. W., Weber, I. T., Bell, G. I., and Pilkis, S. J. (1993).Proc. Natl. Acad. Sci. USA 90 1932–1936.

    Google Scholar 

  • Golshani, S. (1992).Biochem. Med. Metab. Biol. 47 108–115.

    Google Scholar 

  • Gots, R. E., and Bessman, S. P. (1974).Arch. Biochem. Biophys. 163 7–14.

    Google Scholar 

  • Gots, R. E., Gorin, F. A., and Bessman, S. P. (1972).Biochem. Biophys. Res. Commun. 49 1249–1255.

    Google Scholar 

  • Griffin, L. D., Gelb, B. D., Wheeler, D. A., Davison, D., Adams, V., and McCabe, E. R. B. (1991).Genomics 11 1014–1024.

    Google Scholar 

  • Guo, W., Worley, K., Adams, V., Mason, J., Sylvester-Jackson, D., Zhang, Y.-H., Towbin, J. A., Fogt, D. D., Madu, S., Wheeler, D. A., and McCabe, E. R. B. (1993).Nature Genet. 4 367–372.

    Google Scholar 

  • Ha, H., Hajek, P., Bedwell, D., and Burrows, P. (1993).J. Biol. Chem. 268 12143–12149.

    Google Scholar 

  • Hackenbrock, C. R. (1968).Proc. Natl. Acad. Sci. USA 61 598–605.

    Google Scholar 

  • Hattersley, A. T., Turner, R. C., Permutt, M. A., Patel, P., Tanizawa, Y., Chiu, K. C., O'Rahilly, S., Watkins, P. J., and Wainscoat, J. S. (1992).Lancet 339 1307–1310.

    Google Scholar 

  • Hirsch, J. D., Beyer, C. F., Malkowitz, L., Beer, B., and Blume, A. J. (1989).Mol. Pharmacol. 35 157–163.

    Google Scholar 

  • Hoshi, T., Zagotta, W. N., and Aldrich, R. W. (1990).Science 250 533–538.

    Google Scholar 

  • Hurley, J. H., Faber, H. R., Worthylake, D., Meadow, N. D., Roseman, S., Pettigrew, D. W., and Remington, S. J. (1993).Science 259 673–677.

    Google Scholar 

  • Inui, M., and Ishibashi, S. (1979).J. Biochem. 85 1151–1156.

    Google Scholar 

  • Jan, L. Y., and Jan, Y. N. (1989).Cell 56 13–25.

    Google Scholar 

  • Katzen, H. M. (1967).Adv. Enzyme Regul. 5 335–356.

    Google Scholar 

  • Katzen, H. M., and Schimke, R. T. (1965).Proc. Natl. Acad. Sci. USA 54 1218–1225.

    Google Scholar 

  • Kayser, H., Kratzin, H. D., Thinnes, F. P., Göetz, H., Schmidt, W. E., Eckart, K., and Hilschmann, N. (1989).Biol. Chem. Hoppe-Seyler 370 1265–1278.

    Google Scholar 

  • Kerem, B., Rommens, J. M., Buchanan, J. A., Markiewicz, D., Cox, T. K., Chakravarti, A., Buchwald, M., and Tsui, L.-C. (1989).Science 245 1073–1080.

    Google Scholar 

  • Koranyi, L. I., Tanizawa, Y., Welling, C. M., Rabin, D. U., and Permutt, M. A. (1992).Diabetes 41 807–811.

    Google Scholar 

  • Lin, D., Chang, Y. J., Strauss, J. F., III, and Miller, W. L. (1993).Genomics 18 643–650.

    Google Scholar 

  • Lindberg, R. A., Quinn, A. M., and Hunter, T. (1992).Trends Biochem. Sci. 17 114–119.

    Google Scholar 

  • Linden, M., Gellerfors, P., and Nelson, B. D. (1982).FEBS Lett. 141 189–192.

    Google Scholar 

  • Linden, M., Andersson, G., Gellerfors, P., and Nelson, D. B. (1984).Biochim. Biophys. Acta 770 93–96.

    Google Scholar 

  • Magnuson, M. A., Andreone, T. L., Printz, R. L., Koch, S., and Granner, D. K. (1989).Proc. Natl. Acad. Sci. USA 86 4838–4842.

    Google Scholar 

  • Majander, A., Suomalainen, A., Vettenranta, K., Sariola, H., Perkkio, M., Holmberg, C., and Pihko, H. (1991).Pediatr. Res. 30 327–330.

    Google Scholar 

  • Malaisse-Lagae, F., and Malaisse, W. J. (1988).Biochem. Med. Metab. Biol. 39 80–89.

    Google Scholar 

  • Matschinsky, F. M. (1990).Diabetes 39 647–652.

    Google Scholar 

  • McCabe, E. R. B. (1983).Biochem. Med. 30 215–230.

    Google Scholar 

  • McCabe, E. R. B., and Seltzer, W. K. (1986). InMyocardial and Skeletal Muscle Bioenergetics: Proceedings of 2nd International Congress on Myocardial and Cellular Bioenergetics and Compartmentation (Brautbar, N., ed.), Plenum Press, New York, pp. 481–494.

    Google Scholar 

  • McCabe, E. R. B. (1994). InThe Metabolic Basis of Inherited Disease (Scriver, C. R., Beaudet, A. L., Sly, W. S., and Valle, D., eds.), McGraw-Hill, New York, in press.

    Google Scholar 

  • McEnery, M. W., Snowman, A. M., Trifiletti, R. R., and Snyder, S. H. (1992).Proc. Natl. Acad. Sci. USA 89 3170–3174.

    Google Scholar 

  • Miwa, I., Mitsuyama, S., Toyoda, Y., Nonogakii, T., Aoki, S., and Okuda, J. (1990).Biochem. Int. 22 759–767.

    Google Scholar 

  • Moreno-Sanchez, R., Hogue, B. A., Bravo, C., Newman, A. H., Basile, A. S., and Chiang, P. K. (1991).Biochem. Pharmacol. 41 1479–1484.

    Google Scholar 

  • Nelson, B. D., and Kabir, F. (1986).Biochemie 68 407–415.

    Google Scholar 

  • Nishi, S., Seino, S., and Bell, G. I. (1988).Biochem. Biophys. Res. Commun. 157 937–943.

    Google Scholar 

  • Oka, Y., Katagiri, H., Yazaki, Y., Murase, T., and Kobayashi, T. (1993).Lancet 342 527–528.

    Google Scholar 

  • Onisihi, H., Inoue, K., Osaka, H., Kimura, S., Nagatomo, H., Hanihara, T., Kawamoto, S., Okuda, K., Yamada, Y., and Kosaka, K. (1993).J. Neurol. Sci. 114 205–208.

    Google Scholar 

  • Ostlund, A. K., Gohring, U., Krause, J., and Brdiczka, D. (1983).Biochem. Med. 30 231–245.

    Google Scholar 

  • Papadopoulos, V. (1993).Endocrinol. Rev. 14 222–240.

    Google Scholar 

  • Parry, D. M., and Pedersen, P. L. (1983).J. Biol. Chem. 258 10904–10912.

    Google Scholar 

  • Piccolo, G., Aschei, M., Ricordi, A., Banfi, P., Lo Curto, F., and Fratino, P. (1989).J. Neurol. Sci. 94 163–172.

    Google Scholar 

  • Polakis, P. G., and Wilson, J. E. (1985).Arch. Biochem. Biophys. 236 328–337.

    Google Scholar 

  • Quade, A., Zierz, S., and Klingmuller, D. (1992).Clin. Invest. 70 396–402.

    Google Scholar 

  • Reardon, W., Ross, R. J., Sweeney, M. G., Luxon, L. M., Pembrey, M. E., Harding, A. E., and Trembath, R. C. (1992).Lancet 340 1376–1379.

    Google Scholar 

  • Remes, A. M., Majama, K., Herva, R., and Hassinen, I. E. (1993).Neurology 43 1015–1020.

    Google Scholar 

  • Roos, N., Benz, R., and Brdiczka, D. (1982).Biochim. Biophys. Acta 685 202–214.

    Google Scholar 

  • Rotig, A., Cormier, V., Chatelain, P., Francois, R., Saudubray, J. M., Rustin, P., and Munnich, A. (1993).J. Clin. Invest. 91 1095–1098.

    Google Scholar 

  • Sadava, D., Depper, M., Gilbert, M., Bernard, B., and McCabe, E. R. B. (1987).Biol. Neonate 52 26–32.

    Google Scholar 

  • Sargent, C. A., Affara, N. A., Bentley, E., Pelmear, A., Bailey, D. M. D., Davey, P., Dow, D., Leversha, M., Aplin, H., Besley, G. T. N., and Ferguson-Smith, M. A. (1993).Hum. Mol. Genet. 2 97–106.

    Google Scholar 

  • Stoffel, M., Bell, K., Blackburn, C., Powell, K., Seo, T., Takeda, J., Vionnet, N., Xiang, K.-S., Gidh-Jain, M., Pilkis, S., Ober, C. and Bell, G. (1993).Diabetes 42 937–940.

    Google Scholar 

  • Superti-Furga, A., Schoenle, E., Tuchschmid, P., Caduff, R., Sabato, V., DeMattia, D., Gitzelmann, R. and Steinmann, B. (1993).Eur. J. Pediatr. 152 44–50.

    Google Scholar 

  • Thinnes, F. P., Goetz, H., Kayser, H., Benz, R., R., Schmidt, W. E., Kratzin, H. D. and Hilschmann, N. (1989)Biol. Chem. Hoppe-Seyler 370 1253–1264.

    Google Scholar 

  • van den Ouweland, L. M., Lemkes, H. H., Ruitenbeek, W., Sandkuijl, L. A., de Viklder, M. F., Struyvenberg, P. A., van de Kamp, J. J. and Maassen, J. A. (1992).Nature Genet. 1 368–371.

    Google Scholar 

  • Velho, G., Froguel, P., Clement, K., Pueyo, M., Rakotoambinina, B., Zouali, H., Passa, P., Cohen, D. and Robert, J.-J. (1992).Lancet 340 444–448.

    Google Scholar 

  • Vionnet, N., Stoffel, M., Takeda, J., Yasuda, K., Bell, G. I., Zouali, H., Lesage, S., Velho, G., Iris, F., Pass, P., Froguel, P. and Cohen, D. (1992).Nature 356 721–722.

    Google Scholar 

  • Walker, A. P., Muscatelli, F. and Monaco, A. P. (1993).Hum. Mol. Genet. 2 107–114.

    Google Scholar 

  • Walters, E. and McClean, P. (1968).Biochem. J. 109 737–741.

    Google Scholar 

  • Wilson, J. E. (1978).Trends Biochem. Sci. 3 124–125.

    Google Scholar 

  • Wilson, J. E. (1980).Curr. Top. Cell. Regul. 16 1–54.

    Google Scholar 

  • Wilson, J. E. (1985). InRegulation of Carbohydrate Metabolism, Vol. I (Beitner, R., ed.), CRC Press, Cleveland, Ohio, pp. 45–85.

    Google Scholar 

  • Zagotta, W. N., Hoshi, T., and Aldrich, R. W. (1990).Science 250 568–571.

    Google Scholar 

  • Zupanc, M. L., Moraes, C. T., Shanske, S., Langman, C. B., Ciafaloni, E., and DiMauro, S. (1991).Ann. Neurol. 29 680–683.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Baylor College of Medicine, 77030, Houston, Texas

    Edward R. B. McCabe

  2. Department of Molecular and Human Genetics, Baylor College of Medicine, 77030, Houston, Texas

    Edward R. B. McCabe

Authors
  1. Edward R. B. McCabe
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

McCabe, E.R.B. Microcompartmentation of energy metabolism at the outer mitochondrial membrane: Role in diabetes mellitus and other diseases. J Bioenerg Biomembr 26, 317–325 (1994). https://doi.org/10.1007/BF00763103

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00763103

Key words

Search

Navigation