Treatment of Pyruvic and Lactic Acidaemia in Ophthalmoplegia Plus

  • G. Scarlato
  • M. Moggio
  • F. Fici
  • C. Cerri
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

Abstract

Disorders of lactate and pyruvate metabolism are a relatively common feature in a number of different diseases frequently associated with mitochondrial abnormalities in muscle, brain, liver and heart. As it is well-known, pyruvate sits at the center of any ordinary chart of intermediary metabolism. The formation of pyruvate is primarily from glucose by the process of glycolyses and its utilization is by transamination, reduction, carboxylation or oxidation processes. Therefore pyruvate and lactate metabolism are under control of several enzymatic mechanisms.

Keywords

Pyruvic Acid Citric Acid Cycle Mitochondrial Myopathy Mitochondrial Abnormality Lactate Metabolism 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Askanas, V., Engel, W. K., Britton, D. E., Adornato, B. T., and Eiben, R. M., 1978, Reincarnation in cultured muscle of mitochondria abnormalities, Arch. Neurol. 35: 801.Google Scholar
  2. Bastiaensen, L. A. K., Jaspar, H. M. J., Veerkamp, J. H., Brookelman, H., and Von Hinsbergh, V. W. M., 1978, Ophthalmoplegia plus, a real nosological entity, Acta Neurol. Scand., 58: 9CrossRefGoogle Scholar
  3. Bradley, W. G., Tomlinson, B. E., and Hardy, M., 1978, Further studies of mitochondrial and lipid storage myopathies, J. Neurol. Scie. 35: 201.Google Scholar
  4. Cerretelli, S., Marconi, C., and Sassi, G., 1981, The effects of pyridoxine alphaketoglutarate on human maximal aerobic and anaerobic performance, Europ. J. Physiol. in press.Google Scholar
  5. Czok, R., Lamprecht, W., 1974, in “Methoden der Enzymatischen Analyse”, Verlag Chemie, Weinheim p 1491.Google Scholar
  6. Mauro, S., Schotland, D. L., Lee, C. D., Bonilla, E., and Conn, H. L., 1973a, Biochemical and ultrastructural studies of mitochondria in Luft disease: implications for “mitochondrial myopathies”, Trans. Ann. Neurol. Assoc. 97: 265Google Scholar
  7. Mauro, S., Schotland, D. L., Bonilla, E., Lee, C. P., Gambetti, P., Rowland, L. P., 1973b, Progressive ophthalmoplegia, glycogen storage and abnormal mitochondria, Arch. Neurol. 29: 170.Google Scholar
  8. Engel, A. G., Angelini, C., 1973, Carnitine deficiency of human skeletal muscle with associated lipid storage myopathy: a new syndrome. Science 179: 899.PubMedCrossRefGoogle Scholar
  9. Ernster, I., Ikkos, D., and Luft, R., 1959, Enzymatic activities of human skeletal muscle mitochondria: a tool in clinical metabolic research, Nature 184: 1951.Google Scholar
  10. French, J. H., Sheraed, E. S., Lubell, H., Brotz, M. and Moore, C. L., 1972, Trichcopoliodystrophy: report of a case and biochemical studies, Arch. Neurol. 26: 229.Google Scholar
  11. Gonatas, N. K., Evangelista, I., and Martin, J., 1967, A generalized disorder of nervous system, skeletal muscle and heart resembling Refsum’s disease and Hurler’s syndrome, Am. J. Med. 42: 169.Google Scholar
  12. Hanzlikova, V., and Schiaffino, S., 1977, Mitochondrial changes in ischemic skeletal muscle, J. Ultrastruct. Res., 60: 121.PubMedCrossRefGoogle Scholar
  13. Haydar, N. A., Hadley, L. C., Afifi, A., Wakid, N., Bolles, S., and Fawaz, K., 1q71, Severe hypermetabolism with primary abnormality of skeletal muscle mitochondria: functional and therapeutic effects of chloramphenicol treatment, Ann. Int. Med. 74: 548.Google Scholar
  14. Heffner, R. R., and Barron, S. A., 1978, The early effects of ischemia upon skeletal muscle mitochondria, J. Neurol. Sci. 38: 295.Google Scholar
  15. Karpati, G., Carpenter, S., Labrisseau, A., and Lafontaine, R., 1973, The Kearns-Shy Syndrome. A multisysem disease with mitochondrial abnormality demonstrated in skeletal muscle and skin, J. Neurol. Sci., 19: 133.PubMedCrossRefGoogle Scholar
  16. Kark, R. A. P., Weinbad, E. C., Blass, D. P., and Engel, W. K., 1972, Oxidative metabolism in small samples of normal and diseased human muscle, Second. Int. Congress on Muscle Diseases Excerpta medica, 99.Google Scholar
  17. Kark, R. A. P., and Buduelli, R. M., 1979, Pyruvate dehydrogenase deficiency in spinocerebellar degenerations Neurology, 29: 126.Google Scholar
  18. Kearns, T. P., and Sayre, G. P., 1958, Retinitis pigmentosa, external ophthalmoplegia and complete heart block, Arch. Ophthal. 60: 280.Google Scholar
  19. Kuroda, Y., Kline, J. J., Sweetman, L., Nylan, W. L. and Groshong T. D., 1979, Abnormal pyruvate and alpha-ketoglutarate dehydrogenase complexes in a patient with lactic acidemia, Pediatr. Res. 13, 928–931.Google Scholar
  20. Luft, R., Ikkos, D., Palmieri, G., Ernster, L., and Afzelius, B., 1962, A case of severe hypermetabolism of nonthyroid origin and a defect in the maintenance of mitochondrial respiratory control. A correlated clinical, biochemical and morphological study, J. Clin. Inv. 41: 1776.CrossRefGoogle Scholar
  21. Markesberry, W. R., 1979, Lactic acidemia, mitochondrial myopathy and basal ganglia calcification, Neurology 29: 1057Google Scholar
  22. Morgan-Hughes, A., Derveniza, P,, Kahn, S. N., London, D. N., Sherrott, R. M., Land, J. M., and Clark, J. B., 1977, A mitochondrial.myopathy characterized by a deficiency in reducible cytochrome b, Brain 100: 617.Google Scholar
  23. Nemni, R., Mitsumoto, A., and Bradley, W. G., 1981, Morphological and biochemical analysis in chronic progessive ophthalmoplegia, J. Neuropathol. Exptl. Neurol., 40: 350.Google Scholar
  24. Noll, F., 1974, in “Methoden der Enzymatischen Analyse”, Bergmeyer ed. Verlag Chemie, Weinheim p 1521.Google Scholar
  25. Reske-Nielsen, E., Lou, H. C., and Lowes, J., 1976, Progressive external ophthalmoplegia. Evidence for generalized mitochondria) disease with a defect in pyruvate metabolism, Acta Ophtalm. 54: 551.Google Scholar
  26. Sahgal, V., Subramani, V., Hughes, R., Shah, A., and Singh, H., 1979, On the pathogenesis of mitochondrial myopathies. An experimental study, Acta Neuropath. 46: 177.CrossRefGoogle Scholar
  27. Scarlato, G., Pellegrini, G., and Veichsteinas, A., 1978, Morphologic and metabolic studies in a case of oculo-craniosomatic neuromuscular disease, J. Neuropathol. 37: 1.CrossRefGoogle Scholar
  28. Schapira, Y., Cederbaum, S. D, Cancilla, P. A., Nielsen, D., and Lippe, B. M., 1975, Familial poliodystrophy, mitochondrial myopathy and lactate acidemia, Neurology 25: 614.Google Scholar
  29. Schneck, L., Adachi, H., Briet, M., Walintz, A., and Volk, B. W., 1973, Ophthalmoplegia plus with morphological and chemical studies of cerebellar and muscle tissue, J. Neurol. Sci., 19: 37.PubMedCrossRefGoogle Scholar
  30. Spiro, A. J., Moore, C. L., Prineas, J. M., Strasberg, P. M., and Rapin, I., 1970, A cytochrome-related inherited disorder of the nervous system and muscle, Arch. Neurol. 23: 103.Google Scholar
  31. Stumpf, D. A., Mc Cabe, E. R. B., Parks, J. K., and Bullon, W. W., 1979, A mechanism for hyperammonemia in mitochondrial disorders, Neurology, 29: 576.CrossRefGoogle Scholar
  32. Sulaiman, W. K., Doyle, D., Johnson, R. H., and Jannett, S., 1974, Myopathy with mitochondrial inclusion bodies: histological and metabolic studies, J. Neurol. Neurosurg. Psychiat. 37: 1236.Google Scholar
  33. Wijngaarden, G. K., Bethlem, J., Meijer, A. E. F. H., Hulsman, W. C., and Feltkamp, C. A., 1967, Skeletal muscle disease with abnormal mitochondria, Brain 90: 577.Google Scholar

Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • G. Scarlato
    • 1
  • M. Moggio
    • 1
  • F. Fici
    • 2
  • C. Cerri
    • 1
  1. 1.Clinica Neurologica IIa Universita’ di MilanoMilanoItaly
  2. 2.Istituto Lusofarmaco d’ItaliaMilanoItaly

Personalised recommendations