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Immunohistochemical Analysis of Amyloid β-Protein Isoforms in CAA

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Cerebral Amyloid Angiopathy in Alzheimer’s Disease and Related Disorders

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Abstract

Cerebral amyloid angiopathy (CAA) is an amyloid deposition in the wall of arteries and veins in the leptomeninges, and also in the arterioles and capillaries in the cortex. Major isoforms were Aβx−40 (Aβ40) and Aβx−42(43) (Aβ42). Immunohistochemical analysis revealed focal deposits of Aβ42 in the early stages of CAA in both meningeal and cortical vessels. In advanced stages of CAA, the entire vessel wall is labeled for both Aβ40 and Aβ42, and Aβ40 labeling is predominant. In advanced CAA, Aβ40 becomes partly soluble and diffuses out from the vessel walls after dipping the tissue sections in formic acid. The N-terminal of deposited Aβ varies due to proteolytic cleavage and processing. These findings are consistent in normal aged people and in disorders having CAA; i.e.sporadic AD, early-onset familial AD with both APP and presenilin mutations, Down syndrome and HCHWA-D. In an early stage, deposition of amyloid-associated proteins in the basement membrane precedes focal Aβ42 deposition. Initial Aβ42 deposits may be a seed for further massive deposition of Aβ40 (leading to advanced CAA) in both meningeal and cortical vessels.

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Author information

Authors and Affiliations

  1. Gunma University School of Health Sciences, Japan

    Haruyasu Yamaguchi

  2. Department of Neurology of Leiden, University Medical Center Leiden, The Netherlands

    Marion L. C. Maat-Schieman

Authors
  1. Haruyasu Yamaguchi
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  2. Marion L. C. Maat-Schieman
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Editor information

Editors and Affiliations

  1. Departments of Neurology and Pathology, University Medical Center St. Radboud, Nijmegen, The Netherlands

    Marcel M. Verbeek

  2. Department of Pathology, University Medical Center St. Radboud, Nijmegen, The Netherlands

    Robert M. W. de Waal

  3. UCLA Medical Center, Los Angeles, CA, USA

    Harry V. Vinters

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© 2000 Springer Science+Business Media Dordrecht

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Yamaguchi, H., Maat-Schieman, M.L.C. (2000). Immunohistochemical Analysis of Amyloid β-Protein Isoforms in CAA. In: Verbeek, M.M., de Waal, R.M.W., Vinters, H.V. (eds) Cerebral Amyloid Angiopathy in Alzheimer’s Disease and Related Disorders. Springer, Dordrecht. https://doi.org/10.1007/978-94-017-1007-7_10

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  • DOI: https://doi.org/10.1007/978-94-017-1007-7_10

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-90-481-5480-7

  • Online ISBN: 978-94-017-1007-7

  • eBook Packages: Springer Book Archive

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