Fas and fasl expression in leukocytes from chronic granulomatous disease patients. Report as inadecuate




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Daniela Montes-Berrueta ; Lorena Ramírez ; Lisbeth Berrueta ;Investigación Clínica 2012, 53 2

Author: Siham Salmen

Source: http://www.redalyc.org/articulo.oa?id=372937688005


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Investigación Clínica ISSN: 0535-5133 riclinicas@gmail.com Universidad del Zulia Venezuela Salmen, Siham; Montes-Berrueta, Daniela; Ramírez, Lorena; Berrueta, Lisbeth Fas and FasL expression in leukocytes from Chronic Granulomatous Disease patients. Investigación Clínica, vol.
53, núm.
2, abril-junio, 2012, pp.
157-167 Universidad del Zulia Maracaibo, Venezuela Available in: http:--www.redalyc.org-articulo.oa?id=372937688005 How to cite Complete issue More information about this article Journals homepage in redalyc.org Scientific Information System Network of Scientific Journals from Latin America, the Caribbean, Spain and Portugal Non-profit academic project, developed under the open access initiative Invest Clin 53(2): 157 - 167, 2012 Fas and FasL expression in leukocytes from Chronic Granulomatous Disease patients. Daniela Montes-Berrueta, Lorena Ramírez, Siham Salmen and Lisbeth Berrueta. Institute of Clinical Immunology, University of Los Andes.
Mérida, Venezuela. Key words: CGD, Fas-FasL, T lymphocytes, neutrophils. 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 f...





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