Abnormal B cell differentiation and variable increased T cell suppression in immunodeficiency with hyper-IgM.
In vitro immunoglobulin (Ig) synthesis was evaluated in three young men having immunodeficiency with hyper-IgM. Patient and normal T and B cells were separated and cultured in various combinations. 35S-methionine incorporation into Ig was measured using immunoprecipitation, and Ig classes were determined by SDS-polyacrylamide gel electrophoresis with autoradiography. The patient B cells were able to produce only IgM in culture with either autologous or allogeneic T cells. Normal B cells produced IgM, G and A when cultured with normal T cells. T cells from two of the patients suppressed the Ig synthesis of normal B cells; irradiation of these T cells allowed them to provide T helper function. T cells from the third patient expressed normal T suppressor/helper activity. This implies that defective differentiation of B cells into IgG- and IgA-producing plasma cells may be a constant feature of immunodeficiency with hyper-IgM, and that excessive T suppressor activity is a variable accompanying abnormality.
D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology*; D002454:Cell Differentiation; D002478:Cells, Cultured; D002648:Child; D004406:Dysgammaglobulinemia / Q000276:immunology*; D004591:Electrophoresis, Polyacrylamide Gel; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology*; D017098:IgA Deficiency; D017099:IgG Deficiency; D007075:Immunoglobulin M / Q000096:biosynthesis; D008297:Male; D014176:Protein Biosynthesis; D013601:T-Lymphocytes / Q000276:immunology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology*
D007075:Immunoglobulin M