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PMID: 16612983 Published · ppublish English Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Review

The role of cystatin C in cerebral amyloid angiopathy and stroke: cell biology and animal models.

Brain pathology (Zurich, Switzerland) ·Vol. 16 ·No. 1 ·2006-01-00 ·Pages 60-70

Levy E, Jaskolski M, Grubb A

Abstract

A variant of the cysteine protease inhibitor, cystatin C, forms amyloid deposited in the cerebral vasculature of patients with hereditary cerebral hemorrhage with amyloidosis, Icelandic type (HCHWA-I), leading to cerebral hemorrhages early in life. However, cystatin C is also implicated in neuronal degenerative diseases in which it does not form the amyloid protein, such as Alzheimer disease (AD). Accumulating data suggest involvement of cystatin C in the pathogenic processes leading to amyloid deposition in cerebral vasculature and most significantly to cerebral hemorrhage in patients with cerebral amyloid angiopathy (CAA). This review focuses on cell culture and animal models used to study the role of cystatin C in these processes.

MeSH Terms
Alzheimer Disease/genetics Animals Cerebral Amyloid Angiopathy/genetics,pathology Cystatin C Cystatins/biosynthesis,genetics Disease Models, Animal Humans Neurofibrils/genetics Stroke/genetics,pathology
Chemicals
CST3 protein, human Cystatin C Cystatins
Authors & Affiliations
3 authors, click to expand affiliations / ORCID
Levy Efrat
Department of Psychiatry, New York University School of Medicine, and Nathan Kline Institute, Orangeburg 10962, USA. [email protected]
Jaskolski Mariusz
Grubb Anders
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Article Info
Journal
Brain pathology (Zurich, Switzerland)
Abbr.
Brain Pathol
ISSN
1015-6305
Published
2006-01-00
Pages
60-70
Language
English
Region
Switzerland
NLM ID
9216781
PMCID
PMC8095742
Subset
IM
Grants
NIA NIH HHS · AG017617 · United States
NINDS NIH HHS · NS42029 · United States
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