Fine Structure of Early Tay-Sachs Disease

  • M. Adachi
  • J. Torii
  • L. Schneck
  • B. W. Volk
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 19)


Tay-Sachs disease is an inborn error of glycolipid. It is characterized by massive accumulation of GM2-ganglioside (4), mainly in the brain and by the absence of the enzyme hexosaminidase A (5). Despite the significant increase of our knowledge of the biochemical aspects of this disorder during the past few years, the ultrastructural alterations of the neuronal organelles related to the accumulation and the evolution of the intracytoplasmic deposited lipid material at the early stage of Tay-Sachs disease are unknown.


Kupffer Cell Luxol Fast Blue Marked Dilatation Axonal Fiber Cerebral Neuron 
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.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Adachi, M., Torii, J., Karvounis, P.C., and Volk, B.W.: Alterations of Astrocytic Organelles in Various Lipidoses and Allied Diseases. Acta Neuropath., 18, 74, 1971.PubMedCrossRefGoogle Scholar
  2. 2.
    Adachi, M., Torii, J., Schneck, L., and Volk, B.W.: The Fine Structure of Fetal Tay-Sachs Disease. Arch. Path., 91, 48, 1971.PubMedGoogle Scholar
  3. 3.
    Adachi, M., Volk, B.W., Schneck, L., and Torii, J.: Fine Structure of the Myenteric Plexus in Various Lipidoses. Arch. Path., 87, 228, 1969.PubMedGoogle Scholar
  4. 4.
    Klenk, E., Vater, W., and Bartsch, G.: Storage of Gangliosides in Nervous Tissue in Tay-Sachs Disease and Changes in Material Preserved in Formalin. J. Neurochem., 1, 203, 1957.PubMedCrossRefGoogle Scholar
  5. 5.
    Okada, S., and O’Brien, J.S.: Tay-Sachs Disease: General Absence of a B-D-N-Acetyl Hexosaminidase Component. Science, 165, 698, 1969.PubMedCrossRefGoogle Scholar
  6. 6.
    Rouser, G., Kritchesky, G., and Yamamoto, A.: Lipid Chromatographic Analysis. Marinetti, G.V. (Ed.), New York; M. Dekker, Inc., 1967, p. 99.Google Scholar
  7. 7.
    Suzuki, K.: The Pattern of Mammalian Brain Gangliosides: II. Evaluation of the Extraction Procedures, Postmortem Changes and the Effect of Formalin Preservation. J. Neurochem., 12, 629, 1965.PubMedCrossRefGoogle Scholar
  8. 8.
    Wallace, B.J., Volk, B.W., and Lazarus, S.S.: Fine Structural Localization of Acid Phosphatase Activity in Neurons of Tay-Sachs Disease. J. Neuropath. Exp. Neurol., 23, 676, 1964.PubMedCrossRefGoogle Scholar
  9. 9.
    Wallace, B.J., Volk, B.W., Schneck, L., and Kaplan, H.: Fine Structural Localization of Two Hydrolytic Enzymes in the Cerebellum of Children With Lipidoses. J. Neuropath. Exp. Neurol., 25, 76, 1966.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1972

Authors and Affiliations

  • M. Adachi
    • 1
  • J. Torii
    • 1
  • L. Schneck
    • 1
  • B. W. Volk
    • 1
  1. 1.Isaac Albert Research Institute of the Kingsbrook Jewish Medical CenterBrooklynUSA

Personalised recommendations