Document Detail

Tissue-specific renin-angiotensin system in pulmonary lymphangioleiomyomatosis.
MedLine Citation:
PMID:  16474096     Owner:  NLM     Status:  MEDLINE    
Lymphangioleiomyomatosis (LAM), a multisystem disease found in middle-aged women, is characterized by cystic lung destruction and abdominal tumors (e.g., angiomyolipomas, lymphangioleimyomas), resulting from proliferation of abnormal-appearing, smooth muscle-like cells (LAM cells). The LAM cells, in combination with other cells, form nodular structures within the lung interstitium and in the walls of the cysts. LAM cells contain mutations in the tuberous sclerosis complex TSC1 and/or TSC2 genes, which lead to dysregulation of the mammalian target of rapamycin, affecting cell growth and proliferation. Proliferation and migration of vascular smooth muscle cells and production of angiogenic factors are regulated, in part, by angiotensin II. To determine whether a LAM-specific renin-angiotensin system might play a role in the pathogenesis of LAM, we investigated the expression of genes and gene products of this system in LAM nodules. mRNA for angiotensinogen was present in RNA isolated by laser-captured microdissection from LAM nodules. Angiotensin I-converting enzyme and chymase-producing mast cells were present within the LAM nodules. We detected renin in LAM cells, as determined by the presence of mRNA and immunohistochemistry. Angiotensin II type 1 and type II receptors were identified in LAM cells by immunohistochemistry and immunoblotting of microdissected LAM nodules. Angiotensin II is localized in cells containing alpha-smooth muscle actin (LAM cells). A LAM-specific renin-angiotensin system appears to function within the LAM nodule as an autocrine system that could promote LAM cell proliferation and migration, and could represent a pharmacologic target.
Julio C Valencia; Gustavo Pacheco-Rodriguez; Adriana K Carmona; Janina Xavier; Patrick Bruneval; William K Riemenschneider; Yoshihiko Ikeda; Zu-Xi Yu; Victor J Ferrans; Joel Moss
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't     Date:  2006-02-10
Journal Detail:
Title:  American journal of respiratory cell and molecular biology     Volume:  35     ISSN:  1044-1549     ISO Abbreviation:  Am. J. Respir. Cell Mol. Biol.     Publication Date:  2006 Jul 
Date Detail:
Created Date:  2006-06-19     Completed Date:  2006-08-08     Revised Date:  2013-06-07    
Medline Journal Info:
Nlm Unique ID:  8917225     Medline TA:  Am J Respir Cell Mol Biol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  40-7     Citation Subset:  IM    
Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA.
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MeSH Terms
Actins / metabolism
Angiotensin II / metabolism
Angiotensinogen / genetics
Base Sequence
Lung / metabolism*,  pathology*
Lymphangioleiomyomatosis / genetics*,  pathology,  physiopathology
Mast Cells / ultrastructure
Molecular Sequence Data
Organ Specificity
Peptidyl-Dipeptidase A / metabolism
RNA, Messenger / genetics,  metabolism
Receptor, Angiotensin, Type 1 / metabolism
Receptor, Angiotensin, Type 2 / metabolism
Renin / genetics,  metabolism
Renin-Angiotensin System / genetics*
Reg. No./Substance:
0/Actins; 0/RNA, Messenger; 0/Receptor, Angiotensin, Type 1; 0/Receptor, Angiotensin, Type 2; 11002-13-4/Angiotensinogen; 11128-99-7/Angiotensin II; EC A; EC

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