Fas and FasL expression in leukocytes from Chronic Granulomatous Disease patients

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