Journal of Inherited Metabolic Disease

, Volume 7, Issue 3, pp 143–144 | Cite as

In vitro activation of the pyruvate dehydrogenase complex in human muscle by a broad specificity protein phosphatase

  • K. Toshima
  • Y. Kuroda
  • E. Takeda
  • T. Watanabe
  • M. Ito
  • E. Naito
  • M. Miyao
  • I. Nonaka
  • K. Tamagawa
  • T. Toyofuku
  • T. Yamamoto
JSIMD Meeting

Keywords

Public Health Internal Medicine Pyruvate Metabolic Disease Specificity Protein 
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.
This is a preview of subscription content, log in to check access.

References

  1. Blass, J. P. In Burman, D., Holton, J. B. and Pennock, C. A. (eds.)Inherited Disorders of Carbohydrate Metabolism, MTP Press, Lancaster, 1980, pp. 239–268Google Scholar
  2. Blass, J. P., Cederbaum, S. D. and Kark, R. A. P. Rapid diagnosis of pyruvate and ketoglutarate dehydrogenase deficiencies in platelet-enriched preparations from blood.Clin. Chim. Acta 75 (1977) 21–30PubMedGoogle Scholar
  3. DeVivo, D. C., Haymond, M. W., Obert, K. A., Nelson, J. S. and Pagliara, A. S. Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (Leigh's disease).Ann. Neurol. 6 (1979) 483–494PubMedGoogle Scholar
  4. Evans, O. B. Human muscle pyruvate dehydrogenase activity.Neurology (NY) 33 (1983) 1463–1471Google Scholar
  5. Harris, R. A., Paxton, R. and Parker, R. A. Activation of the branched-chain α-ketoacid dehydrogenase complex by a broad specificity protein phosphatase.Biochem. Biophys. Res. Commun. 107 (1982) 1497–1503PubMedGoogle Scholar
  6. Reed, L. J. Multienzyme complexes.Accts. Chem. Res. 7 (1974) 40–46Google Scholar
  7. Sheu, K. F. R., Hu, C. W. C. and Utter, M. F. Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts.J. Clin. Invest. 67 (1981) 1463–1471PubMedGoogle Scholar
  8. Toshima, K., Kuroda, Y., Hashimoto, T., Ito, M., Watanabe, T., Miyao, M. and Ii, K. Enzymologic studies and therapy of Leigh's disease associated with pyruvate decarboxylase deficiency.Pediatr. Res. 16 (1982) 430–435PubMedGoogle Scholar
  9. Wieland, O. H., Siess, E., Schultze-Wethmar, F. H., von Funcke, H. G. and Winton, B. Active and inactive forms of pyruvate dehydrogenase in rat heart and kidney: effect of diabetes, fasting and refeeding on pyruvate dehydrogenase interconversion.Arch. Biochem. Biophys. 143 (1971) 593–601PubMedGoogle Scholar

Copyright information

© SSIEM and MTP Press Limited 1984

Authors and Affiliations

  • K. Toshima
    • 1
  • Y. Kuroda
    • 1
  • E. Takeda
    • 1
  • T. Watanabe
    • 1
  • M. Ito
    • 1
  • E. Naito
    • 1
  • M. Miyao
    • 1
  • I. Nonaka
    • 2
  • K. Tamagawa
    • 3
  • T. Toyofuku
    • 3
  • T. Yamamoto
    • 3
  1. 1.Department of PediatricsUniversity of Tokushima School of MedicineTokushimaJapan
  2. 2.Division of Neuromuscular ResearchNational Center for Nervous, Mental and Muscular DisordersTokyoJapan
  3. 3.Tokyo Metropolitan Neurological HospitalTokyoJapan

Personalised recommendations