Fas and FasL expression in leukocytes from Chronic Granulomatous Disease patients / Expresión de Fas y FasL en leucocitos de pacientes con Enfermedad Granulomatosa Crónica
Invest. clín
;
53(2): 157-167, jun. 2012. ilus
Artigo
em Inglês
| LILACS
| ID: lil-664574
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.
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Proteína Ligante Fas
/
Doença Granulomatosa Crônica
/
Leucócitos
Limite:
Adolescente
/
Adulto
/
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Invest. clín
Assunto da revista:
Biologia
/
Medicina
/
Relatos de Casos
Ano de publicação:
2012
Tipo de documento:
Artigo
País de afiliação:
Venezuela
Instituição/País de afiliação:
University of Los Andes/VE
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