Uncoupling of T-cell antigen receptor and downstream protein tyrosine kinases in common variable immunodeficiency.
Clin Immunol Immunopathol
; 84(1): 98-102, 1997 Jul.
Article
em En
| MEDLINE
| ID: mdl-9191889
ABSTRACT
Patients with common variable immunodeficiency (CVID) are heterogeneous in the clinical manifestations of the disease and the underlying mechanisms leading to the immunodeficiency. Although the overt defect is an impairment in B-cell function, there is increasing evidence of primary T-cell dysfunctions in a proportion of patients with CVID. We have analyzed T-cells from six CVID patients for activation of both early and late events in response to TCR triggering. The data showed that T-cells from three of six CVID patients were defective in the capacity to initiate the TCR/CD3 signaling pathway by activating intracellular tyrosine kinases, associated with impaired proliferative responses to TCR/CD3 triggering. Since both surface expression of the TCR/CD3 complex and intracellular expression of key tyrosine kinases such as p56lek and ZAP-70 were normal in these patients, our data suggest a defect in the earliest step of TCR signal transduction.
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Base de dados:
MEDLINE
Assunto principal:
Proteínas Tirosina Quinases
/
Linfócitos T
/
Imunodeficiência de Variável Comum
Idioma:
En
Ano de publicação:
1997
Tipo de documento:
Article