Patterns of Brain Ganglioside Fatty Acids in Sphingolipidoses

  • B. Berra
  • V. Zambotti
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 25)


In the last few years the development of new methods of analyses resulted in an improvement in our knowledge of lipidoses; the accumulated lipids were better characterized and very often the enzymatic defect of the disease was determined. In many cases however, only the carbohydrate moiety of the accumulated glycolipids was studied, while the lipid moiety, particularly as far as the ganglioside fatty acids distribution is concerned, was poorly investigated. The data in the literature on this topic are very few; Rosemberg (1) investigated the pattern of fatty acids in Tay-Sachs’ disease, but his determinations were made on the gangliosides mixture, and not on the single ganglioside. The fatty acid distribution of grey matter GM1 in GM1 gangliosidosis, type I, was reported by Suzuki (2); the fatty acid composition of nine ganglioside pure fractions in subacute sclerosing leuko-encephalitis was reported by Ledeen et al. (3); recently Eto et al. (4) gave the fatty acid composition of gangliosides GM3, GM2, GM1, GD1a in white matter in a case of Krabbe’s disease.


Fatty Acid Composition Grey Matter Fatty Acid Distribution Trace Trace Brain Ganglioside 
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  1. (1).
    Rosemberg, A., in Inborn Disorders of Sphingolipid Metabolism, Ed. S. M. Aronson and B.W. Volk, Pergamon Press, New York, p. 267 (1967).Google Scholar
  2. (2).
    Suzuki, K. and Kamoshita, S., J. Neuropath. & Exptl. Neurol. 28: 25 (1969).Google Scholar
  3. (3).
    Ledeen, R., Salsman, K. and Cabrera, M., J. Lipid Res. 9: 129 (1968).Google Scholar
  4. (4).
    Eto, Y. and Suzuki, K., J. Neurochem. 18: 503 (1971).CrossRefGoogle Scholar
  5. (5).
    Trams, E.G. and Lauter, C. J., Biochim. Biophys. Acta 60: 350 (1962).CrossRefGoogle Scholar
  6. (6).
    Tettamanti, G., Bertona, L., Berra, B. and Zambotti, V., Ital. J. Biochem. 13: 315 (1964).Google Scholar
  7. (7).
    Bonali, F., Tettamanti, G. and Cervato, G., Metabolismo 5: 269 (1969).Google Scholar
  8. (8).
    Matturri, L., Coggi, G., Berra, B. and Bonali, F., II Morgagni 1: 297 (1969).Google Scholar
  9. (9).
    Galli, G., White, H. B. Jr. and Paoletti, R., J. Neurochem. 17: 347 (1970).CrossRefGoogle Scholar
  10. (10).
    O’Brien, J. S., Okada, S., Wan Ho, M., Fillerup, D. L., Veath, M. L., Adams, K., in Lipid Storage Disease, Ed. J. Bernsohn and J. H. Grossman, Academic Press, New York, p. 225 (1971).Google Scholar
  11. (11).
    Van Bogaert, L., in Maladies nerveuses génétiques d’ordre métabolique, Rev. Méd. de Liége, Univ. de Liége, p. 17(1962).Google Scholar
  12. (12).
    Siakotos, A.N. and Rouser, G., J. Amer. Oil Chem. Soc. 42: 913 (1965).CrossRefGoogle Scholar
  13. (13).
    Zeman, W. and Donahue, S., Acta Neuropath. 3: 144 (1963).CrossRefGoogle Scholar
  14. (14).
    Berra, B., Coggi, G. and Matturri, L., in preparation.Google Scholar

Copyright information

© Plenum Press, New York 1972

Authors and Affiliations

  • B. Berra
    • 1
  • V. Zambotti
    • 1
  1. 1.Department of BiochemistryMedical School University of MilanoItaly

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