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The Cerebral β-Amyloid Angiopathies: Hereditary and Sporadic

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Zhang-Nunes, Sandy X., Maat-Schieman, Marion L. C., Duinen, Sjoerd G., Roos, Raymund A. C., Frosch, Matthew P., Greenberg, Steven M.·2006·Brain Pathology

Abstract

We review the clinical, radiologic, and neuropathologic features of the hereditary and sporadic forms of cerebral amyloid angiopathy (CAA) associated with vascular deposition of the beta-amyloid peptide. Amino acid substitutions at 4 sites in the beta-amyloid precursor protein, all situated within the beta-amyloid peptide sequence itself, have been shown to cause heritable forms of CAA. The vascular diseases caused by these mutations are associated primarily with cerebral hemorrhages, white matter lesions, and cognitive impairment, and only variable extents of the plaque and neurofibrillary pathologies characteristic of Alzheimer disease. Sporadic CAA typically presents 20 or more years later than hereditary CAA, but is otherwise characterized by a comparable constellation of recurrent cerebral hemorrhages, white matter lesions, and cognitive impairment. The clinical, radiologic and pathologic similarities between hereditary and sporadic CAA suggest that important lessons for this common age-related process can be learned from the mechanisms by which mutation makes beta-amyloid tropic or toxic to vessels.

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Metadata

Authors
Zhang-Nunes, S. X., Maat-Schieman, M. L. C., Duinen, S. G., Roos, R. A. C., Frosch, M. P., & Greenberg, S. M.
Venue
Brain Pathology
Publisher
Wiley
Published
2006-01
Volume
16
Issue
1
Pages
30-39
ISSN
  • 1015-6305 (ISSN-L)
  • 1750-3639
OpenAlex
W2005695389
Cited by
193
References
24
FWCI
11.50
Type
journal article
Language
en
Open access
green
Licence
doi.wiley.com/10.1002/tdm_license_1 (tdm, 3530 d)

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CoChat. (2026). Citation check: The Cerebral β-Amyloid Angiopathies: Hereditary and Sporadic. https://app.cochat.ai/papers/10.1111/j.1750-3639.2006.tb00559.x
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Zhang-Nunes, S. X., Maat-Schieman, M. L. C., Duinen, S. G., Roos, R. A. C., Frosch, M. P., & Greenberg, S. M. (2006). The Cerebral β-Amyloid Angiopathies: Hereditary and Sporadic. Brain Pathology. https://doi.org/10.1111/j.1750-3639.2006.tb00559.x

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