Histochemical and Ultrastructural Study of Gaucher Cells

  • M. Elleder
  • A. Jirásek
Part of the Acta Neuropathologica Supplementum book series (NEUROPATHOLOGIC, volume 7)


Findings are presented of the histochemical and electron microscopical studies of several bioptic specimens of a juvenile form of Gaucher’s disease. The storage was restricted to the macrophages with the exception of occasional hepatocytes in which structures closely resembling the storage tubules were found. The cytoplasmic striations of GC were found to be composed both of lipid and a glycoprotein components which can be easily dissociated from each other by means of either organic solvent extraction or proteolytic digestion. According to the results obtained it is the glycoprotein component which is mainly responsible for the cytoplasmic striations. On the contrary the majority of the tubular storage material of the Krabbe cells was found to be removed with chloroform methanol. Results of the enzyme histochemical study (hydrolases and dehydrogenases) point to the considerably higher overall metabolic activity of GC when compared to the Niemann-Pick storage macrophages.

Key words

Gaucher cells macrophages ultra-structure histochemistry glycoproteins storage 


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  1. 1.
    Elleder M (1975) The unusually high activity of mitochondrial α-glycerophosphate dehydrogenase in Gaucher cells. Virchows Archiv [Cell Pathol] 19:97–99Google Scholar
  2. 2.
    Elleder M, Šmíd F (1977) Lysosomal non-lipid component of Gaucher’s cells. Virchows Archiv [Cell Pathol] 26:133–138Google Scholar
  3. 3.
    Elleder M, Šmíd F, Harzer K, Čihula J (1980) Niemann-Pick disease. Analysis of liver tissue in sphingomyelinase-deficient patients. Virchows Archiv [Pathol Anat] 385:215–231CrossRefGoogle Scholar
  4. 4.
    Fisher ER, Reidbord H (1962) Gaucher’s disease: pathogenetic considerations based on electron microscopic and histochemical observations. Am J Pathol 41:679–692PubMedGoogle Scholar
  5. 5.
    Friedman RB, Kanfer JN (1974) Composition of sialoglycans isolated from Gaucher’s spleen. Biochem Med 9:327–333PubMedCrossRefGoogle Scholar
  6. 6.
    Kanfer JN, Stein M, Spielvogel C (1972) Recent observations on Gaucher’s disease. In: Volk BW, Aronson SM (eds) Sphingolipids, sphingolipidoses and allied disorders. Plenum Press, New York, pp 225–236Google Scholar
  7. 7.
    Lennert K (1978) Malignant lymphomas. Lennert K (ed) Springer, Berlin Heidelberg New York, p 55Google Scholar
  8. 8.
    Lojda Z, Gossrau R, Schiebler TH (1979) Enzyme histochemistry. Springer, Berlin Heidelberg New YorkCrossRefGoogle Scholar
  9. 9.
    Nelson E, Aurebeck G, Osterberg K, Jabbour JT (1963) Ultrastructural and chemical studies on Krabbe’s disease. J Neuropathol Exp Neurol 22:414–434PubMedCrossRefGoogle Scholar
  10. 10.
    O’Brien JS (1978) The gangliosidoses. In: Stanbury JB, Wyngaarden JB, Fredrickson DS (eds) The metabolic basis of inherited disease. McGraw-Hill, New York, pp 841–865Google Scholar
  11. 11.
    Suzuki K (1970) Ultrastructural study of experimental globoid cells. Lab Invest 23:612–619PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1981

Authors and Affiliations

  • M. Elleder
    • 1
    • 2
  • A. Jirásek
    • 1
  1. 1.Hlava 1st Department of Pathology, Medical FacultyCharles UniversityPragueCSSR
  2. 2.1st Department of PathologyFaculty of MedicinePrague 2ČSSR

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