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Vascular accumulation of the small leucine-rich proteoglycan decorin in CADASIL.
MedLine Citation:
PMID:  25026535     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Small penetrating brain artery thickening is a major feature of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Although affected fibrotic arteries of CADASIL have been shown to accumulate collagen, other components that compose pathological arterial walls remain incompletely characterized. We investigated the expression of decorin (DCN), the first collagen-binding small leucine-rich proteoglycan identified, in CADASIL. DCN was markedly upregulated in pathologically affected leptomeningeal and small penetrating arteries in CADASIL and was notably weaker in normal arteries from control brains. DCN protein was localized principally to the media and adventitia and only occasionally expressed in the intima. Immunoblotting of brain lysates showed a three-fold increase of DCN in CADASIL brains (compared with controls). Messenger RNA encoding DCN was five-fold increased in CADASIL. We conclude that DCN is the first identified proteoglycan to be identified in CADASIL arteries and may accumulate through transcriptional mechanisms. Additional studies are warranted to determine whether DCN localizes broadly to pathological small vessels in other cerebrovascular disorders.
Authors:
Soo Jung Lee; Xiaojie Zhang; Michael M Wang
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-7-14
Journal Detail:
Title:  Neuroreport     Volume:  -     ISSN:  1473-558X     ISO Abbreviation:  Neuroreport     Publication Date:  2014 Jul 
Date Detail:
Created Date:  2014-7-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9100935     Medline TA:  Neuroreport     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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