Document Detail

Pediatric lung disease: from proteinases to pulmonary fibrosis.
MedLine Citation:
PMID:  15786437     Owner:  NLM     Status:  MEDLINE    
One distinctive outcome of interstitial lung diseases in childhood is the abnormal accumulation of pulmonary extracellular matrix. The clinical consequence of such excessive connective tissue accumulation is known as pulmonary fibrosis. While numerous aspects of its pathogenesis have become familiar, many key events involved in its inception and progression still remain unclear. There is now compelling evidence that lung damage due to uncontrolled proteolysis may help drive critical processes that regulate fibrotic matrix remodeling. In this regard, a number of proteinases have been implicated in promoting both the initial lung injury and the fibroproliferative repair that follows. This review summarizes the knowledge of how different matrix-targeting enzymes may act to influence the development of pediatric pulmonary fibrosis. Understanding the scientific basis of this complex process may highlight opportunities to limit unwanted proteolysis and the intensity of its fibrotic sequelae.
Felix Chua; Peter D Sly; Geoffrey J Laurent
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Pediatric pulmonology     Volume:  39     ISSN:  8755-6863     ISO Abbreviation:  Pediatr. Pulmonol.     Publication Date:  2005 May 
Date Detail:
Created Date:  2005-04-04     Completed Date:  2005-08-02     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  392-401     Citation Subset:  IM    
Copyright Information:
Copyright 2005 Wiley-Liss, Inc
Centre for Respiratory Research, Royal Free and University College London Medical School, Rayne Institute, London, UK.
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MeSH Terms
Connective Tissue / enzymology
Extracellular Matrix / enzymology
Lung Diseases, Interstitial / enzymology*,  etiology
Peptide Hydrolases / physiology*
Pulmonary Alveoli / enzymology
Pulmonary Fibrosis / enzymology*,  etiology
Reg. No./Substance:
EC 3.4.-/Peptide Hydrolases

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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