%0 Journal Article
%T Fas and FasL expression in leukocytes from Chronic Granulomatous Disease patients
%A Montes-Berrueta
%A Daniela
%A Ram¨ªrez
%A Lorena
%A Salmen
%A Siham
%A Berrueta
%A Lisbeth
%J Investigaci¨®n Cl¨ªnica
%D 2012
%I Scientific Electronic Library Online
%X 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
%K cgd
%K fas/fasl
%K t lymphocytes
%K neutrophils
%K egc
%K fas/fasl
%K linfocitos t
%K neutr¨®filos.
%U http://www.scielo.org.ve/scielo.php?script=sci_abstract&pid=S0535-51332012000200005&lng=en&nrm=iso&tlng=en