Other neurodegenerative conditions I

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Introduction Amyloid is a proteinaceous substance made of fibrils that are shown by electron microscopy to be 8–10 nm wide and are composed of insoluble peptides. In all types of amyloid, the peptides, which derive from precursor proteins, change their secondary structure into β-sheet-rich structures. The accumulation of amyloid may occur extracellularly or intracellularly. In the brain, extracellular amyloid may appear as deposits in the parenchyma (amyloid plaques) or within the vessel wall (cerebral amyloid angiopathy [CAA]). Cerebral amyloid angiopathy is the neuropathological hallmark of biochemically and genetically diverse disorders characterized by the presence of amyloid in the walls of arteries and, in some cases, in capillaries of the brain parenchyma and leptomeninges [1–4]. This accumulation may represent the failure of vessels in eliminating soluble forms of amyloidogenic peptides from the brain [4]. The amyloidogenic peptides found in cases of CAA are listed in Table 13.1. Among CAAs, amyloid-β (Aβ) CAA (Aβ-CAA) is the most common and is frequently present in sporadic and genetically determined Alzheimer’s disease (AD) and in some forms of hereditary cerebral hemorrhage with amyloidosis (HCHWA), and may also occur in non-demented individuals [1, 2, 5]. Cerebral amyloid angiopathy is also found in association with mutations in the prion protein (PRNP), BRI2, transthyretin (TTR), cystatin C (CST3) and gelsolin (GSN) genes [2, 6–11]. Here, we will describe the most significant clinicopathological characteristics of these CAAs, classified according to the amyloid protein involved.

Original languageEnglish (US)
Title of host publicationNeuropathology of Neurodegenerative Diseases: A Practical Guide
PublisherCambridge University Press
Pages249-258
Number of pages10
ISBN (Print)9781107588660, 9781107674202
DOIs
StatePublished - Jan 1 2014

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Cerebral Amyloid Angiopathy
Amyloid
Peptides
Familial Cerebral Amyloid Angiopathy
Brain
Gelsolin
Amyloidogenic Proteins
Cystatin C
Prealbumin
Protein Precursors
Amyloid Plaques
Electron Microscopy
Alzheimer Disease
Arteries
Mutation
Genes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Vidal, R., & Ghetti, B. (2014). Other neurodegenerative conditions I. In Neuropathology of Neurodegenerative Diseases: A Practical Guide (pp. 249-258). Cambridge University Press. https://doi.org/10.1017/CBO9781107588660.013

Other neurodegenerative conditions I. / Vidal, Ruben; Ghetti, Bernardino.

Neuropathology of Neurodegenerative Diseases: A Practical Guide. Cambridge University Press, 2014. p. 249-258.

Research output: Chapter in Book/Report/Conference proceedingChapter

Vidal, R & Ghetti, B 2014, Other neurodegenerative conditions I. in Neuropathology of Neurodegenerative Diseases: A Practical Guide. Cambridge University Press, pp. 249-258. https://doi.org/10.1017/CBO9781107588660.013
Vidal R, Ghetti B. Other neurodegenerative conditions I. In Neuropathology of Neurodegenerative Diseases: A Practical Guide. Cambridge University Press. 2014. p. 249-258 https://doi.org/10.1017/CBO9781107588660.013
Vidal, Ruben ; Ghetti, Bernardino. / Other neurodegenerative conditions I. Neuropathology of Neurodegenerative Diseases: A Practical Guide. Cambridge University Press, 2014. pp. 249-258
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