Resumo

MONTES-BERRUETA, Daniela; RAMIREZ, Lorena; SALMEN, Siham  e  BERRUETA, Lisbeth. Fas and FasL expression in leukocytes  from Chronic Granulomatous Disease patientsExpresión de Fas y FasL en leucocitos de pacientes con Enfermedad Granulomatosa Crónica. Invest. clín [online]. 2012, vol.53, n.2, pp.157-167. ISSN 0535-5133.

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

Palavras-chave : CGD; Fas/FasL; T lymphocytes; neutrophils; EGC; Fas/FasL; linfocitos T; neutrófilos.