Document Detail

Evidence for a functional role of the molecular chaperone clusterin in amyloidotic cardiomyopathy.
MedLine Citation:
PMID:  21224044     Owner:  NLM     Status:  MEDLINE    
Molecular chaperones, including the extracellular protein clusterin (CLU), play a significant role in maintaining proteostasis; they have a unique capacity to bind and stabilize non-native protein conformations, prevent aggregation, and keep proteins in a soluble folding-competent state. In this study, we investigated amyloid-infiltrated cardiac tissue for the presence of CLU and measured serum levels of CLU in patients with and without amyloidotic cardiomyopathy (CMP). Cardiac tissues containing amyloid deposits composed of either transthyretin (TTR) or Ig light chain from nine patients with amyloidotic CMP were examined for the presence of CLU using immunohistochemical techniques. CLU staining coincided with the extracellular myocardial amyloid deposits in tissues from patients with familial TTR, senile systemic, and Ig light chain amyloidosis. The association of CLU with cardiac amyloid deposits was confirmed by immunogold electron microscopy. Serum concentrations of CLU were measured in familial TTR, senile systemic, and Ig light chain amyloidosis patient groups and compared with both age-matched healthy controls and with patients with CMP unrelated to amyloid disease. Subset analysis of disease cohorts, based on cardiac involvement, indicated that decreased serum CLU concentrations were associated with amyloidotic CMP. Taken together, these results suggest that CLU may play a pathogenetic role in TTR and Ig light chain amyloidoses and amyloidotic CMP.
Michael J Greene; Flora Sam; Pamela T Soo Hoo; Rupesh S Patel; David C Seldin; Lawreen H Connors
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2010-12-23
Journal Detail:
Title:  The American journal of pathology     Volume:  178     ISSN:  1525-2191     ISO Abbreviation:  Am. J. Pathol.     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-01-12     Completed Date:  2011-04-18     Revised Date:  2013-07-02    
Medline Journal Info:
Nlm Unique ID:  0370502     Medline TA:  Am J Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  61-8     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2011 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.
Department of Pathology, Boston University School of Medicine, Boston, Massachusetts, USA.
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MeSH Terms
Aged, 80 and over
Amyloidosis, Familial / metabolism*,  pathology
Cardiomyopathies / metabolism*,  pathology
Clusterin / analysis,  metabolism*
Immunoglobulin Light Chains / analysis,  metabolism
Middle Aged
Molecular Chaperones / analysis,  metabolism*
Prealbumin / analysis,  metabolism
Grant Support
Reg. No./Substance:
0/CLU protein, human; 0/Clusterin; 0/Immunoglobulin Light Chains; 0/Molecular Chaperones; 0/Prealbumin

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