Studies for gene on immunodeficients' B cells

点击翻译按钮获取中文摘要

Immunological and genetic studies were carried out for immunodeficients' B cells.Common variable immunodeficiency (CVI) is a heterogeneous group of diseases. Firstly, B cell function was investigated using pokeweed mitogen induced immunaglobulin production system and Staphylococcus aureus Cowan I plus recombinant interleukin 2 on CVI patients. B cells from 6 patients with CVI were disturbed in the process of activation, proliferation and differentiation. Secondary, structure of immunoglobulin (Ig) gene was investigated using Ig probe and Southern blot analysis for germ line DNA of patients. No deletion and mutation was found in patients' IgH gene. Thirdly, expression of Ig gene was investigated using northern blot analysis for lymphocytes of patients. In most patients, each class of Ig gene was not expressed. However, in a part of patients, C mu gene was expressed, but C gamma gene was not expressed. B cell lines were prepared by EB virus. Rearrangement of Ig gene w as investigated for patients' B cell lines. In some cases, Ig genes have been rearranged, whereas in the other cases Ig genes have not been rearranged. Moreover, c-myc gene was expressed in some patients, but not in the other patients.From our results, it became clear that there is heterogeneity in the pathogenesis of immunodeficiency (especially, a- or hypo-gammaglobulinemia). It is abnormality in activation of B cells, rearrangement of Ig gene, or expression of Ig gene.