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Microglia is a component of the prion protein amyloid plaque in the Gerstmann-Sträussler-Scheinker syndrome

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Summary

The microglial cell has been demonstrated as component of the cerebral amyloid plaque of Alzheimer's disease. Involvement of microglia in plaques of another cerebral amyloidosis, the Gerstmann-Sträussler-Scheinker syndrome (GSS), has found little attention. We examine here the presence of microglia in GSS plaques by immunohistochemistry and transmission electron microscopy. Paraffin sections from five brains of patients with GSS were immunolabelled with antibodies against prion protein, A4/β amyloid protein, ferritin, leukocyte common antigen, HLA-DR, CD 68, and the MAC387 epitope for microglia and monocytes/macrophages; microglia was also labelled with the Ricinus communis agglutinin-1 lectin. Such (immuno)labelling demonstrated many delicate cell processes and occasional somata within and around prion protein plaques in all GSS brains. Microglial immunoreactivity was strongest with anti-ferritin and variable with anti-macrophage antibodies. Ultrastructural examination of brain tissue from one autopsy and one biopsy of GSS identified microglial cells in close proximity of amyloid plaque fibrils. Our observations demonstrate microglia as an important component of the amyloid plaque in GSS and suggest a major role for microglia in processing and deposition, or at least organization, of prion protein amyloid. Thus, plaques in both transmissible and nontransmissible cerebral amyloidoses seem to develop via similar pathogenetic mechanisms, irrespective of differences in etiology and molecular composition of the amyloid.

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Authors and Affiliations

  1. Department of Neuropathology, Medical Research Center, Polish Academy of Sciences, Warsaw, Poland

    M. Barcikowska

  2. Electron Microscopic Laboratory, Department of Oncology, Medical Academy Lodz, Poland

    P. P. Liberski

  3. Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA

    P. P. Liberski, P. Brown & D. C. Gajdusek

  4. Institute for Brain Research, University of Tübingen, Germany

    J. W. Boellaard

  5. Neurological Institute, University of Vienna, Schwarzspanierstrasse 17, A-1090, Wien, Austria

    H. Budka

Authors
  1. M. Barcikowska
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  2. P. P. Liberski
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  3. J. W. Boellaard
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  4. P. Brown
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  5. D. C. Gajdusek
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  6. H. Budka
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Additional information

Supported in part by the Austrian Fund for the Advancement of Scientific Research (P8196-MED). This study is part of the EC BIOMED I Project “The human prion diseases” led by H. Budka

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Barcikowska, M., Liberski, P.P., Boellaard, J.W. et al. Microglia is a component of the prion protein amyloid plaque in the Gerstmann-Sträussler-Scheinker syndrome. Acta Neuropathol 85, 623–627 (1993). https://doi.org/10.1007/BF00334672

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  • DOI: https://doi.org/10.1007/BF00334672

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