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Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles

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Abstract

Muscles from themdx mouse (X-linked genetic disorder similar to Duchenne muscular dystrophy) lack dystrophin-associated transsarcolemmal proteins1 and show reduced maintenance metabolic rates2. Here, microcalorimetric comparisons of metabolic stimulation by exogenous substrates in isolated muscles revealed substrate-selective limitation of chemical reaction rates through both glycolytic and TCA-cycle pathways, identical in slow- and fast-twitchmdx muscles. This systemic approach, as opposed to comparisons of single-enzyme activities, sheds new light on the function of dystrophin and associated proteins. The in vivo efficiency of metabolic pathways may depend on stabilization of enzyme complexes by dystrophin-associated elements of the cytoskeleton.

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References

  1. Ervasti, J. M., and Campbell, K. P., Cell66 (1991) 1121.

    Article  CAS  PubMed  Google Scholar 

  2. Decrouy, A., Even, P. C., and Chinet, A., Experientia49 (1993) 843.

    Article  CAS  PubMed  Google Scholar 

  3. Vignos, P. J., and Lefkowitz, M., J. clin. Invest.38 (1959) 873.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Younkin, D. P., Berman, P., Sladky, J., Chee, C., Bank, W., and Chance, B., Neurology37 (1987) 165.

    Article  CAS  PubMed  Google Scholar 

  5. Barbiroli, B., Funicello, R., Lotti, S., Montagna, P., Ferlini, A., and Zaniol, P., J. neurol. Sci.109 (1992) 188.

    Article  CAS  PubMed  Google Scholar 

  6. Dunn, J. F., Frostick, S., Brown, G., and Radda, G. K., Biochim. biophys. Acta1096 (1991) 115.

    Article  CAS  PubMed  Google Scholar 

  7. Turner, P. R., Westwood, T., Regen, C. M., and Steinhardt, R. A., Nature335 (1988) 735.

    Article  CAS  PubMed  Google Scholar 

  8. Fong, P., Turner, P. R., Denetclaw, W. F., and Steinhardt, R. A., Science250 (1990) 673.

    Article  CAS  PubMed  Google Scholar 

  9. Franco, A., and Lansman, J. B., Nature344 (1990) 670.

    Article  CAS  PubMed  Google Scholar 

  10. Chinet, A., Decrouy, A., and Even, P. C., J. Physiol.455 (1992) 663.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Barbiroli, B., Funicello, R., Ferlini, A., Montagna, P., and Zaniol, P., Muscle & Nerve15 (1992) 344.

    Article  CAS  Google Scholar 

  12. Dunn, J. F., Tracey, I., and Radda, G. K., J. neurol. Sci.113 (1992) 108.

    Article  CAS  PubMed  Google Scholar 

  13. Stoward, P. J., and Stapleton, A., Histochem. J.19 (1987) 598.

    Article  Google Scholar 

  14. Arnold, H., and Pette, D., Eur. J. Biochem.6 (1968) 163.

    Article  CAS  PubMed  Google Scholar 

  15. Hofer, H. W., Gerlach, G., Birkel, G., and Kirschenlohr, H. L., Biochem. Soc. Trans.15 (1987) 982.

    Article  CAS  PubMed  Google Scholar 

  16. Chen-Zion, M., Bassukevitz, Y., and Beitner, R., Int. J. Biochem.24 (1992) 1661.

    Article  CAS  PubMed  Google Scholar 

  17. Minaschek, G., Gröschel-Stewart, U., Blum, S., and Bereiter-Hahn, J., Eur. J. Cell Biol.58 (1992) 418.

    CAS  PubMed  Google Scholar 

  18. Ervasti, J. M., and Campbell, K. P., Curr. Op. Cell Biol.5 (1993) 82.

    Article  CAS  PubMed  Google Scholar 

  19. Griffith, L. M., and Pollard, T. D., J. Cell Biol.78 (1978) 958.

    Article  CAS  PubMed  Google Scholar 

  20. Ball, E. H., and Singer, S. J., Proc. natl Acad. Sci. USA79 (1982) 123.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Hirokawa, N., J. Cell Biol.94 (1982) 129.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Arakawa, T., and Frieden, C., J. biol. Chem.259 (1984) 11730.

    Article  CAS  PubMed  Google Scholar 

  23. Linden, M., Nelson, B. D., and Leterrier, J.-F., Biochem. J.261 (1989) 167.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Price, M. G., and Gomer, R. H., Cell Motil. Cytoskel.13 (1989) 274.

    Article  CAS  Google Scholar 

  25. Gupta, R. S., TIBS15 (1990) 415.

    PubMed  Google Scholar 

  26. Saetersdal, T., Greve, G., and Dalen, H., Histochemistry95 (1990) 1.

    Article  CAS  PubMed  Google Scholar 

  27. Kano, Y., Fujimaki, N., and Ishikawa, H., Cell Struct. Funct.16 (1991) 251.

    Article  CAS  PubMed  Google Scholar 

  28. Bifulco, M., Laezza, C., Aloj, S. M., and Garbi, C., J. cell. Physiol.155 (1993) 340.

    Article  CAS  PubMed  Google Scholar 

  29. Robinson, J. B., Inman, L., Sumegi, B., and Srere, P. A., J. biol. Chem.262 (1987) 1786.

    Article  CAS  PubMed  Google Scholar 

  30. Sumegi, B., McCammon, M. T., Sherry, A. D., Keys, D. A., McAlister-Henn, L., and Srere, P. A., Biochemistry31 (1992) 8720.

    Article  CAS  PubMed  Google Scholar 

  31. Erecinska, M., and Wilson, D. F., J. Memb. Biol.70 (1982) 1.

    Article  CAS  Google Scholar 

  32. Krebs, H. A., and Johnson, W. A., Enzymologia4 (1937) 148.

    CAS  Google Scholar 

  33. Chinet, A., Experientia46 (1990) 1194.

    Article  CAS  PubMed  Google Scholar 

  34. Brown, G. C., Biochem. J.284 (1992) 1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Kholodenko, B. N., Demin, O. V., and Westerhof, H. V., FEBS Lett.320 (1993) 75.

    Article  CAS  PubMed  Google Scholar 

  36. Small, R., and Kacser, H., Eur. J. Biochem.213 (1993) 75.

    Google Scholar 

  37. Förster, M. E. C., J. theor. Biol.133 (1988) 1.

    Article  PubMed  Google Scholar 

  38. McLennan, P. A., McArdle, A., and Edwards, R. H. T., Biochem. J.275 (1991) 477.

    Article  Google Scholar 

  39. Chi, M. M.-Y., Hintz, C. S., McKee, D., Felder, S., Grant, N., Kaiser, K. K., and Lowry, O. H., Metabolism36 (1987) 761.

    Article  CAS  PubMed  Google Scholar 

  40. Lilling, G., and Beitner, R., Biochem. Med. metab. Biol.45 (1991) 319.

    Article  CAS  PubMed  Google Scholar 

  41. Glesby, M. J., Rosenmann, E., Nylen, E. G., and Wrogemann, K., Muscle & Nerve11 (1988) 852.

    Article  CAS  Google Scholar 

  42. Daut, J., and Elzinga, G., J. Physiol.413 (1989) 379.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Phillips, S. K., Wiseman, R. W., Woledge, R. C., and Kushmerick, M. J., J. Physiol.462 (1993) 135.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Chew, S. F., and Ip, Y. K., Comp. Biochem. Physiol.104B (1993) 681.

    CAS  Google Scholar 

  45. Wikman-Coffelt, J., Stefenelli, T., Wu, S. T., Parmley, W. W., and Jasmin, G., Circulation Res.68 (1991) 45.

    Article  CAS  PubMed  Google Scholar 

  46. Adams, V., Griffin, L., Towbin, J., Gelb, B., Worley, K., and McCabe, E. R. B., Biochem. Med. metab. Biol.45 (1991) 271.

    Article  CAS  PubMed  Google Scholar 

  47. Wallimann, T., Wyss, M., Brdiczka, D., Nicolay, K., and Eppenberger, H., Biochem. J.281 (1992) 21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Strickland, J. M., and Ellis, D. A., Nature253 (1975) 464.

    Article  CAS  PubMed  Google Scholar 

  49. Cassens, R. G., Bocek, R. M., and Beatty, C. H., Am. J. Physiol.217 (1969) 715.

    Article  CAS  PubMed  Google Scholar 

  50. Mayr, G. W., Eur. J. Biochem.143 (1984) 513.

    Article  CAS  PubMed  Google Scholar 

  51. Wrogemann, K. J., and Pena, S. D., Lancet1 (1976) 672.

    Article  CAS  PubMed  Google Scholar 

  52. Wiche, G., Briones, K., Koszka, C., Artlieb, U., and Krepler, R., EMBO J.3 (1984) 991.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  53. Niggli, V., and Burger, M. M., J. Membr. Biol.100 (1987) 97.

    Article  CAS  PubMed  Google Scholar 

  54. Danowski, B. A., Imanaka-Yoshida, K., Sanger, J. M., and Sanger, J., J. Cell Biol.118 (1992) 1411.

    Article  CAS  PubMed  Google Scholar 

  55. Straub, V., Bittner, R. E., Léger, J. L., and Voit, T., J. Cell Biol.119 (1992) 1183.

    Article  CAS  PubMed  Google Scholar 

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Chinet, A.E., Even, P.C. & Decrouy, A. Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles. Experientia 50, 602–605 (1994). https://doi.org/10.1007/BF01921731

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