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.
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Herzig, M.C., Winkler, D.T., Walker, L.C., Jucker, M. (2001). Transgenic Mouse Models of Cerebral Amyloid Angiopathy. In: Tolnay, M., Probst, A. (eds) Neuropathology and Genetics of Dementia. Advances in Experimental Medicine and Biology, vol 487. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1249-3_10
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DOI: https://doi.org/10.1007/978-1-4615-1249-3_10
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