Document Detail


Fas and FasL expression in leukocytes from chronic granulomatous disease patients.
MedLine Citation:
PMID:  22978048     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
Abstract. Chronic Granulomatous Disease (CGD) is a primary immunodeficiency characterized by defects in superoxide (O2-) production, which result from mutations in one of the four NADPH oxidase components, predisposing to bacterial and fungal infections. Besides the O2-defect, it has been described that neutrophils from CGD patients are resistant to cell death, a phenomenon that has been connected to chronic inflammation and predisposition to autoimmune diseases. A diminished expression of Fas and its counterpart FasL, molecules known to play a major role in cell death, has been described in lymphocytes depleted of O2-reactive oxygen species (ROS), suggesting an involvement of ROS in Fas/FasL expression. In this work, Fas and FasL expressions were analyzed in T cells and neutrophils from two CGD families, previously known to harbor two different molecular defects: absence of either p47-phox or p67-phox. We found that T lymphocytes from CGD patients express low levels of Fas and FasL, while a diminished FasL expression was observed on neutrophils from a CGD A470 patient. These defects may contribute to understand altered cell death in CGD patients.
Authors:
Daniela Montes-Berrueta; Lorena Ramírez; Siham Salmen; Lisbeth Berrueta
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Investigación clínica     Volume:  53     ISSN:  0535-5133     ISO Abbreviation:  Invest Clin     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-09-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0421531     Medline TA:  Invest Clin     Country:  Venezuela    
Other Details:
Languages:  eng     Pagination:  157-67     Citation Subset:  IM    
Affiliation:
Institute of Clinical Immunology, University of Los Andes, Mérida, Venezuela.
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