Skip to main content
SpringerLink
Log in

Pathological Cytosomes

  • Chapter
Handbook of Neurochemistry

Abstract

Several varieties of morphologically abnormal subcellular organelles are found in neurons or glial cells in a number of diseases, particularly the lipid storage disorders. Terry and Korey(1) first described the ultrastructural characteristics of abnormal intraneuronal cytosomes in a patient with infantile GM2-gangliosidosis (Tay-Sachs disease). The morphology of this lamellar structure was subsequently studied in detail.(2) The typical Tay-Sachs membranous cytoplasmic bodies (MCB) are approximately 1 μ in diameter, consisting of concentrically arranged lamellar structures. The periodicity of this lamellar structure is 50 to 60 Å. Often there is a homogeneous or finely granular zone at the center of an MCB. In a fully developed stage, as is usually the case in the cerebral cortex, Tay-Sachs MCB lack the surrounding limiting membrane, and the outermost lamella appears identical with those making up the interior. However, MCB with a surrounding single unit membrane have been observed in the cerebellum of a Tay-Sachs patient.(3) This was thought to be an early stage of MCB development.

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

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. R. D. Terry and S. R. Korey, Membranous cytoplasmic granules in infantile amaurotic idiocy, Nature 188: 1000–1002 (1960).

    Article  PubMed  CAS  Google Scholar 

  2. R. D. Terry and M. Weiss, Studies in Tay-Sachs disease. II. Ultrastructure of the cerebrum, J. Neuropath. Exptl. Neurol. 22: 18–55 (1963).

    Article  CAS  Google Scholar 

  3. B. J. Wallace, S. Schneck, H. Kaplan, and B. W. Volk, Fine structure of the cerebellum of children with lipidoses, Arch. Path. 80: 466–486 (1965).

    PubMed  CAS  Google Scholar 

  4. N. K. Gonatas and J. Gonatas, Ultrastructural and biochemical observations on a case of systemic late infantile lipidosis and its relationship to Tay-Sachs disease and gargoylism, J. Neuropath. Exptl. Neurol 24: 318–340 (1965).

    Article  CAS  Google Scholar 

  5. K. Suzuki, K. Suzuki, and G. C. Chen, Morphological, histochemical, and biochemical studies on a case of systemic late infantile lipidosis (generalized gangliosidosis), J. Neuropath. Exptl. Neurol. 27: 15–38 (1968).

    Article  CAS  Google Scholar 

  6. K. Suzuki, K. Suzuki, I. Rapin, Y. Suzuki, and N. Ishii, Juvenile GM2-gangliosidosis: clinical variant of Tay-Sachs disease or a new disease? Neurology 20: 190–204 (1970).

    PubMed  CAS  Google Scholar 

  7. F. Aleu, R. D. Terry, and H. Zellweger, Electron microscopy of two cerebral biopsies in gargoylism, J. Neuropath. Exptl. Neurol. 24: 304–317 (1965).

    Article  CAS  Google Scholar 

  8. B. W. Volk and B. J. Wallace, The liver in lipidosis: an electron microscopic and histochemical study. Amer. J. Path. 49: 203–225 (1966).

    PubMed  CAS  Google Scholar 

  9. B. J. Wallace, D. Kaplan, M. Adachi, L. Schneck, and B. W. Volk, Mucopolysaccharidosis type III. Morphologic and biochemical studies of two siblings with Sanfilippo syndrome, Arch. Path. 82: 461–473 (1966).

    Google Scholar 

  10. S. Luse, in Inborn Disorders of Sphingolipid Metabolism (S. M. Aronson and B. W. Volk, eds.) pp. 93–105, Pergamon Press, Oxford (1967).

    Google Scholar 

  11. T. v. Hirsch and J. Peiffer, Über Histologische Methoden in der Differential-diagnose von Leucodystrophien und Lipidosen, Arch. Psychiat. Nervenkr. 194: 88–104 (1955).

    Article  Google Scholar 

  12. A. Grégoire, O. Périer, and P. Dustin, Metachromatic leukodystrophy, an electron microscopic study, J. Neuropath. Exptl. Neurol. 25: 617–636 (1966).

    Article  Google Scholar 

  13. W. Zeman and S. Donahue, Fine structure of lipid bodies in juvenile amaurotic idiocy, Acta Neuropath. 3: 144–149 (1963).

    Article  PubMed  CAS  Google Scholar 

  14. N. K. Gonatas, R. D. Terry, R. Winkler, S. R. Korey, C. J. Gomez, and A. Stein, A case of juvenile lipidosis: electron microscopic and biochemical observation of a cerebral biopsy, J. Neuropath. Exptl. Neurol. 22: 557–579 (1963).

    Article  CAS  Google Scholar 

  15. S. Donahue, W. Zeman, and I. Watanabe, in Inborn Disorders of Sphingolipid Metabolism (S. M. Aronson and B. W. Volk, eds.), pp. 3–22, Pergamon Press, Oxford (1967).

    Google Scholar 

  16. K. Suzuki, A. B. Johnson, E. Marquet, and K. Suzuki, A case of juvenile lipidosis: electron microscopic, histochemical and biochemical studies. Acta Neuropath. 11: 122–139 (1968).

    Article  PubMed  CAS  Google Scholar 

  17. P. E. Duffy, M. Kornfeld, and K. Suzuki, Neurovisceral storage disease with curvilinear bodies, J. Neuropath. Exptl. Neurol. 27: 351–370 (1968).

    Article  Google Scholar 

  18. D. L. Odor, R. Janeway, L. A. Pearce, and J. R. Ravens, Progressive myoclonus epilepsy with Lafora inclusion bodies. II Studies of ultrastructure, Arch. Neurol. 16: 583–594 (1967).

    Article  PubMed  CAS  Google Scholar 

  19. S. Samuels, S. R. Korey, J. Gonatas, R. D. Terry, and M. Weiss, Studies in Tay-Sachs disease IV. Membranous cytoplasmic bodies, J. Neuropath. Exptl. Neurol. 22: 81–97 (1963).

    Article  CAS  Google Scholar 

  20. S. Yokoi, J. Austin, F. Witmer, and M. Sakai. Studies in myoclonus epilepsy (Lafora body form). I. Isolation and preliminary characterization of Lafora bodies in two cases, Arch. Neurol. 19: 15–33 (1968).

    Article  PubMed  CAS  Google Scholar 

  21. K. Suzuki, K. Suzuki, and S. Kamoshita, Chemical pathology of GM1-gangliosidosis (generalized gangliosidosis), J. Neuropath. Exptl. Neurol. 28: 25–73 (1969).

    Article  CAS  Google Scholar 

  22. K. Suzuki, K. Suzuki, and G. C. Chen, Isolation and chemical characterization of metachromatic granules from a brain with metachromatic leukodystrophy, J. Neuropath. Exptl. Neurol. 26: 537–549 (1967).

    Article  CAS  Google Scholar 

  23. S. Kamoshita, A. M. Aron, K. Suzuki, and K. Suzuki, Infantile Niemann-Pick disease. A chemical study with isolation and characterization of membranous cytoplasmic bodies and myelin, Amer. J. Dis. Child. 117: 379–394 (1969).

    PubMed  CAS  Google Scholar 

  24. W. T. Norton, S. E. Poduslo, and K. Suzuki, Subacute sclerosing leukoencephalitis. II. Chemical studies including abnormal myelin and an abnormal ganglioside pattern, J. Neuropath. Exptl. Neurol. 25: 582–597 (1966).

    Article  CAS  Google Scholar 

  25. S. Samuels, N. K. Gonatas, and M. Weiss, Formation of the membranous cytoplasmic bodies in Tay-Sachs disease: An in vitro study, J. Neuropath. Exptl. Neurol. 24: 256–264 (1965).

    Article  CAS  Google Scholar 

  26. S. Samuels, N. K. Gonatas, and M. Weiss, Chemical and ultrastructural comparison of synthetic and pathologic membrane system, J. Cell. Biol. 21: 148–152 (1964).

    Article  PubMed  CAS  Google Scholar 

  27. B. J. Wallace, B. W. Volk, and S. S. Lazarus, Fine structural localization of acid phosphatase activity in neurons of Tay-Sachs disease, J. Neuropath. Exptl. Neurol. 23: 676–691 (1964).

    Article  CAS  Google Scholar 

  28. B. J. Wallace, B. W. Volk, L. Schneck, and H. Kaplan. Fine structural localization of two hydrolytic enzymes in the cerebellum of children with lipidoses, J. Neuropath. Exptl. Neurol. 25: 76–96 (1966).

    Article  CAS  Google Scholar 

  29. S. Okada and J. S. O’Brien, Generalized gangliosidosis: β-galactosidase deficiency, Science 160: 1002–1004 (1968).

    Article  PubMed  CAS  Google Scholar 

  30. R. O. Brady, J. N. Kanfer, M. B. Mock, and D. S. Frederickson, The metabolism of sphingomyelin: II. Evidence of an enzymatic deficiency in Niemann-Pick disease, Proc. Natl. Acad. Sci. 55: 366–369 (1966).

    Article  PubMed  CAS  Google Scholar 

  31. J. H. Austin, D. McAfee, D. Armstrong, M. Rourke, L. Shearer, and B. K. Bachhawat, Low sulfatase activities in metachromatic leucodystrophy (MLD), Trans. Amer. Neurol. Assoc. 89: 147–150 (1964).

    CAS  Google Scholar 

  32. J. H. Austin, D. McAfee, D. Armstrong, M. Rourke, L. Shearer, and B. K. Bachhawat, Abnormal sulfatase activities in two human diseases (metachromatic leucodystrophy and gargoylism), Biochem. J. 93: 15c–17c (1964).

    PubMed  CAS  Google Scholar 

  33. E. Mehl and H. Jatzkewitz, Evidence for the genetic block in metachromatic leucodystrophy (ML), Biochem. Biophys. Res. Commun. 19: 407–411 (1965).

    Article  PubMed  CAS  Google Scholar 

  34. A. Taghavy, K. Salsman, and R. Ledeen, An abnormal ganglioside pattern from a gargoyle brain, Fed. Proc. 23: 128 (1964).

    Google Scholar 

  35. K. Suzuki, in Inborn Disorders of Sphingolipid Metabolism (S. M. Aronson and B. W. Volk, eds.) pp. 215–230, Pergamon Press, Oxford (1967).

    Google Scholar 

  36. A. Tingey, Results of glycolipid analysis in certain types of lipidosis and leukodystrophy, J. Neurochem. 3: 230–237 (1959).

    Article  PubMed  CAS  Google Scholar 

  37. P. F. Borri, G. J. M. Hooghwinkel, and G. W. F. Edgar, Brain ganglioside pattern in three forms of amaurotic idiocy and gargoylism, J. Neurochem. 13: 1249–1256 (1966).

    Article  PubMed  CAS  Google Scholar 

  38. A. Bignami, G. Palladini, and P. Borri, Neuronal lipidosis with visceral involvement in an adult, Acta Neuropath. 12: 50–61 (1969).

    Article  Google Scholar 

  39. L. Svennerholm, Chromatographic separation of human brain gangliosides, J. Neurochem. 10: 613–623 (1963).

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Departments of Neurology and Pathology (Neuropathology), University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA

    Kunihiko Suzuki & Kinuko Suzuki

Authors
  1. Kunihiko Suzuki
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kinuko Suzuki
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Neurochemistry and Drug Addiction, New York State Research Institute, Ward’s Island, New York, New York, USA

    Abel Lajtha

Rights and permissions

Reprints and permissions

Copyright information

© 1972 Plenum Press, New York

About this chapter

Cite this chapter

Suzuki, K., Suzuki, K. (1972). Pathological Cytosomes. In: Lajtha, A. (eds) Handbook of Neurochemistry. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-7172-8_7

Download citation

  • DOI: https://doi.org/10.1007/978-1-4615-7172-8_7

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4615-7174-2

  • Online ISBN: 978-1-4615-7172-8

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation