Skip to main content
Log in

Lipids of myelin, white matter and gray matter in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation

  • Communications
  • Published:
Lipids

Abstract

The lipid classes and fatty acid compositions of myelin, white matter and gray matter were analyzed in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation. When compared with normal data, the percentage of 24∶1 was considerably decreased and diminished unsaturation was observed in cerebrosides, whereas the sum of 24∶0 and 24∶1 was the same as in normals. The ratio of hydroxy fatty acids to total fatty acids in cerebrosides was low. The contents of cholesterol and phospholipids in white matter were reduced to 80% of the normal, whereas cerebroside was reduced to 48% of the normal.

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

Access this article

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

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Holloway, P.W., and J.T. Katz, Biochemistry 11:3689 (1972).

    Article  PubMed  CAS  Google Scholar 

  2. Leroux, A., C. Junien, and J. Kaplan, Nature 258:619 (1975).

    Article  PubMed  CAS  Google Scholar 

  3. Kaplan, J.C., A. Leroux, S. Bakouri, J.P. Grangaud, and M. Benadbadji, Nouv. Rev. Fr. Hemat. 14:755 (1974).

    CAS  Google Scholar 

  4. Kaplan, J.C., A. Hanzlichova-Leroux, and J.C. Dreyfus, C.R. Acad. Sci. (Paris) 270:2223 (1970).

    CAS  Google Scholar 

  5. Svennerholm, L., M.-T. Vanier, and B. Jungbjier, J. Neurochem. 30:1383 (1978).

    Article  PubMed  CAS  Google Scholar 

  6. Norton, W.T., and S.E. Poduslo, J. Neurochem. 21:749 (1973).

    Article  PubMed  CAS  Google Scholar 

  7. Svennerholm, L., J. Lipid Res. 9:570 (1968).

    PubMed  CAS  Google Scholar 

  8. Vanier, M.T., M. Holm, R. Öhman, and L. Svennerholm, J. Neurochem. 18:581 (1971).

    PubMed  CAS  Google Scholar 

  9. Svennerholm, L., and M.T. Vanier, Brain Res. 9:215 (1972).

    Google Scholar 

  10. Alling, C., Å. Bruce, I. Karlsson, and L. Svennerholm, J. Neurochem. 23:1263 (1974).

    PubMed  CAS  Google Scholar 

  11. Crawford, N., Clin. Chim. Acta 3:357 (1958).

    Article  PubMed  CAS  Google Scholar 

  12. Svennerholm, L., J. Neurochem. 11:839 (1964).

    Article  PubMed  CAS  Google Scholar 

  13. Svennerholm, L., S. Ställberg-Stenhangen, J. Lipid Res. 9:215 (1968).

    PubMed  CAS  Google Scholar 

  14. Gerstl, B., L.F. Eng, M. Tavaststjerna, J.K. Smith, and S.L. Kruse, J. Neurochem. 17:677 (1970).

    Article  PubMed  CAS  Google Scholar 

  15. Johnson, R.V., and S.N. Shah, J. Neurochem. 21:1225 (1973).

    Article  PubMed  CAS  Google Scholar 

  16. Hirono, H., and Y. Wada, J. Nutr. 108:766 (1978).

    PubMed  CAS  Google Scholar 

  17. Murad, S., R.H.K. Chen, and Y. Kishimoto, J. Biol. Chem. 252:5206 (1977).

    PubMed  CAS  Google Scholar 

  18. Bourre, J.M., S. Pollet, M. Paturneau-Jones, and N. Baumann, in “Enzymes of Lipid Metabolism,” Edited by S. Gatt, L. Freysz, and P. Mandel, Plenum Publishing Corporation, New York, 1978, pp. 17–26.

    Google Scholar 

  19. Bourre, J.M., H.H. Bornhofen, C.A. Araoz, O. Daudu, and N.A. Baumann, J. Neurochem. 30:719 (1977).

    Article  Google Scholar 

  20. Ozols, J. Ann. Clin. Res. 8:suppl. 17:182 (1976).

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

About this article

Cite this article

Hirono, H. Lipids of myelin, white matter and gray matter in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation. Lipids 15, 272–275 (1980). https://doi.org/10.1007/BF02535839

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02535839

Keywords

Navigation