IMR Press / FBL / Volume 18 / Issue 4 / DOI: 10.2741/4173

Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.

Open Access Review

Hereditary and sporadic beta-amyloidoses

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1 Division of Neurology 5 - Neuropathology, IRCCS Foundation, Carlo Besta Neurological Institute, Via Celoria 11, 20133 Milan, Italy
Academic Editor:Roberta Ghidoni
Front. Biosci. (Landmark Ed) 2013, 18(4), 1202–1226; https://doi.org/10.2741/4173
Published: 1 June 2013
Abstract

Cerebral amyloidoses are chronic, progressive neurodegenerative diseases that are caused by the aggregation and deposition of misfolded proteins in the central nervous system, and lead to cognitive deficits, stroke, and focal neurological dysfunction including cerebellar and extrapyramidal signs. Among them, betaamyloidoses are a heterogenous set of conditions characterised by the deposition of beta-amyloid protein in brain parenchyma and/or vessel walls that lead to the development of two main clinico-pathological entities: Alzheimer’s disease and cerebral amyloid angiopathy, which may be sporadic or familial, and may also coexist in the same patient. The aim of this review is to describe the most important differences in the pathways leading to parenchymal and cerebrovascular betaamyloidoses, and the main clinical, neuropathological and biochemical characteristics of the two conditions. It also discusses the phenotypes associated with a series of familial and sporadic beta-amyloidoses in more detail in order to highlight the clinical and neuropathological features that may help to distinguish the different forms of disease.

Keywords
Amyloid
Abeta
Alzheimer’s Disease
Amyloidosis
Cerebral Amyloid Angiopathy
CAA
Presenilin
Amyloid Precursor Protein
APP
dementia
Cerebral Hemorrage
Autosomal Recessive App Mutation
Review
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