Transgenic Mouse Models of Cerebral Amyloid Angiopathy

  • Martin C. Herzig
  • David T. Winkler
  • Lary C. Walker
  • Mathias Jucker
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 487)

Abstract

The likelihood of amyloid deposition in the cerebral vasculature (cerebral amyloid angiopathy; CAA) increases with age1-3. Particularly in Alzheimer’s disease (AD), CAA can be detected in up to 90% of the patients4-6. CAA of the ß-amyloid-type (Aß-CAA) affects primarily leptomeningeal and cortical vessels and is associated with degeneration of smooth muscle cells and endothelial cells, as well as blood-brain barrier damage. Severe Aß-CAA can cause fatal cerebral hemorrhage and increases the risk of cerebral microinfarcts7-9. Genetic variations that are causative or risk factors for Aß-CAA have been identified, such as mutations in the genes for ß-amyloid precursor protein (APP), presenilins 1 and 2, and possibly cystatin C, as well as polymorphisms in apolipoprotein E3. In normal aging and AD, Aß-CAA is associated with parenchymal Aß plaques. However, Aß-CAA can also occur in the absence of plaques, as evidenced in hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D)10-12.

Keywords

Codon Dementia Cysteine Neurol Amyloidosis 

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Copyright information

© Springer Science+Business Media New York 2001

Authors and Affiliations

  • Martin C. Herzig
    • 1
  • David T. Winkler
    • 1
  • Lary C. Walker
    • 2
  • Mathias Jucker
    • 1
  1. 1.Department of NeuropathologyInstitute of Pathology, University of BaselBaselSwitzerland
  2. 2.Neuroscience TherapeuticsPfizer Ann Arbor Laboratories, Ann ArborUSA

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