title: Fas and FasL expression in leukocytes from chronic granulomatous disease patients.
abstract: 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.affiliation: Institute of Clinical Immunology, University of Los Andes, Mérida, Venezuela.
authors.forename: Daniela
authors.initials: D
authors.lastname: Montes-Berrueta
authors.forename: Lorena
authors.initials: L
authors.lastname: Ramírez
authors.forename: Siham
authors.initials: S
authors.lastname: Salmen
authors.forename: Lisbeth
authors.initials: L
authors.lastname: Berrueta
mesh_terms: D000293:Adolescent; D000328:Adult; D053222:Fas Ligand Protein / Q000096:biosynthesis*; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000378:metabolism*; D006801:Humans; D007962:Leukocytes / Q000378:metabolism*; D008297:Male; D055815:Young Adult; D019014:fas Receptor / Q000096:biosynthesis*
chemical_list: C505661:FASLG protein, human; D053222:Fas Ligand Protein; D019014:fas Receptor