Impaired TH2 response in patients with Vav1-deficient common variable immunodeficiency with T-cell defects.

摘要

Common variable immunodeficiency (CVID) is a primary immune disorder including a heterogeneous group of diseases with the unifying feature of hypogammaglobulinemia.1 Defects not only in B cells, which are directly responsible for antibody production, but also in other immune cells involved in the generation of an effective humoral response, including antigen-presenting cells and T_H cells, represent the molecular basis of CVIDT_H cells orchestrate the host immune response and can be divided into different subsets based on their pattern of cytokine secretion. T_H1 cells produce IFN-gamma and TNF-beta, which elicit mac-rophage activation and cell-mediated immunity, whereas T_H2 cells produce IL-4 and IL-13, which promote B-cell maturation and antibody production. The differentiation of T_hI and T_H2 subsets is finely regulated by specific transcription factors, cytokines, and costimulatory signals. Among the lineage-specific transcription factors, T-box transcription factor (T-bet) plays a central role in T_H1 cell development, whereas GATA-3 and c-Maf drive T_H2 cell differentiation.