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Amyloidogenic Proteins in Human Central Nervous System Diseases

  • Chapter
Amyloidosis

Abstract

The amyloid proteins which are deposited in the central nervous system fall into two major categories: first, as an involvement incidental to the occurrence of the various forms of systemic amyloidosis; second, amyloid deposits which are restricted to the central nervous system (Table I). This essay is concerned with the latter category, but it is important to consider the implication of this division of nervous system amyloidoses: in the systemic amyloidoses, deposition occurs in the brain in areas where there is an inherently increased permeability of the blood-brain barrier; in contrast, the amyloidoses which are restricted to the brain have a rather distinctive parenchymal and vascular distribution, suggesting that the origin of the amyloid protein (or its precursor) is not hematogenous. It will become apparent later that we favor the concept of a neuronal origin of these brain-restricted amyloidoses, although it is admitted at the start that much of this concept is still hypothetical and speculative.

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Author information

Authors and Affiliations

  1. Laboratory of Molecular and Applied Neuropathology, Neuromuscular Research Institute, Western Australia, 6009, Australia

    Colin L. Masters

  2. Department of Pathology, University of Western Australia, Western Australia, 6009, Australia

    Colin L. Masters

  3. Department of Neuropathology, Royal Perth Hospital, Western Australia, 6001, Australia

    Colin L. Masters

  4. Institute of Genetics University of Cologne, Cologne, Federal Republic of Germany

    Konrad Beyreuther

Authors
  1. Colin L. Masters
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  2. Konrad Beyreuther
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Editor information

Editors and Affiliations

  1. Department of Internal Medicine, Div of Immunochemistry, University Hospital Groningen, 59 Oostersingel, 9713 EZ, Groningen, The Netherlands

    Jan Marrink Ph.D.

  2. Department of Internal Medicine, Div of Rheumatology, University Hospital Groningen, Oostersingel 59, 9713 EZ, Groningen, The Netherlands

    Martin H. Van Rijswijk M.D.

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© 1986 Martinus Nijhoff Publishers, Dordrecht

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Masters, C.L., Beyreuther, K. (1986). Amyloidogenic Proteins in Human Central Nervous System Diseases. In: Marrink, J., Van Rijswijk, M.H. (eds) Amyloidosis. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-4309-4_17

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  • DOI: https://doi.org/10.1007/978-94-009-4309-4_17

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-8415-4

  • Online ISBN: 978-94-009-4309-4

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