Document Detail

Early pathogenesis of cardiac amyloid deposition in senile systemic amyloidosis: close relationship between amyloid deposits and the basement membranes of myocardial cells.
MedLine Citation:
PMID:  12647215     Owner:  NLM     Status:  MEDLINE    
Despite a number of in vitro studies of transthyretin (TTR) amyloidogenesis the early stage of in vivo amyloidogenesis in the human heart is largely unknown. A heart with a mild degree of cardiac amyloidosis removed from a 90-year old woman at autopsy was selected for analysis. The genotype of the TTR was the wild type. An immunohistochemical study with anti-TTR antibody was performed on serial paraffin sections, and 17 TTR-positive lesions less than 50 micro m in diameter consisting of 13 interstitial and 4 vascular lesions were identified. The early interstitial lesions start as thick membranous deposits between interfacing myocardial cells. They are Congophilic with green birefringence and positive for apolipoprotein E but negative for amyloid P component. The TTR-positive amyloid extends along intercellular spaces and becomes larger, involving several myocardial fibers. The media is the initial site of arteriolar involvement. According to the in vitro studies of amyloid fibrillogenesis, the most critical step is formation of the nucleus under supersaturated conditions. The supersaturated conditions are speculated to be achieved by binding to proteoglycans or lipid membranes. Our results indicate that the basement membrane of myocardial cells is the initial site of amyloid deposition, providing a suitable place for concentration of TTR.
Motoji Sawabe; Akihiko Hamamatsu; Tateki Ito; Tomio Arai; Kumiko Ishikawa; Kouji Chida; Naotaka Izumiyama; Naoko Honma; Kaiyo Takubo; Masamitsu Nakazato
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Publication Detail:
Type:  Journal Article     Date:  2003-02-26
Journal Detail:
Title:  Virchows Archiv : an international journal of pathology     Volume:  442     ISSN:  0945-6317     ISO Abbreviation:  Virchows Arch.     Publication Date:  2003 Mar 
Date Detail:
Created Date:  2003-03-20     Completed Date:  2003-05-09     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9423843     Medline TA:  Virchows Arch     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  252-7     Citation Subset:  IM    
Departments of Pathology and Cardiology, Tokyo Metropolitan Geriatric Hospital, 35-2 Sakae-cho, Itabashi, 173-0015, Tokyo, Japan.
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MeSH Terms
Aged, 80 and over
Amyloid / analysis*
Amyloidosis / metabolism*,  pathology
Basement Membrane / pathology*
Heart Diseases / metabolism*,  pathology
Microscopy, Electron
Myocardium / chemistry*,  ultrastructure
Prealbumin / analysis,  genetics
Reg. No./Substance:
0/Amyloid; 0/Prealbumin

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