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伴有高IgM的免疫缺陷中异常的B细胞分化及可变增加的T细胞抑制。

Abnormal B cell differentiation and variable increased T cell suppression in immunodeficiency with hyper-IgM.

作者信息

Krantman H J, Stiehm E R, Stevens R H, Saxon A, Seeger R C

出版信息

Clin Exp Immunol. 1980 Apr;40(1):147-56.

PMID:6446430
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1536961/
Abstract

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.

摘要

对三名患有高IgM免疫缺陷的年轻男性进行了体外免疫球蛋白(Ig)合成评估。分离患者及正常的T细胞和B细胞,并以各种组合进行培养。使用免疫沉淀法测定掺入Ig中的35S-甲硫氨酸,并通过SDS-聚丙烯酰胺凝胶电泳及放射自显影法确定Ig类别。患者B细胞在与自体或异体T细胞共培养时仅能产生IgM。正常B细胞与正常T细胞共培养时可产生IgM、G和A。两名患者的T细胞抑制正常B细胞的Ig合成;对这些T细胞进行照射后,它们能够发挥T辅助功能。第三名患者的T细胞表现出正常的T抑制/辅助活性。这意味着B细胞向产生IgG和IgA的浆细胞的分化缺陷可能是高IgM免疫缺陷的一个恒定特征,而过度的T抑制活性是一个可变的伴随异常情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a002/1536961/c9e83f40ee5f/clinexpimmunol00193-0160-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a002/1536961/c9e83f40ee5f/clinexpimmunol00193-0160-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a002/1536961/c9e83f40ee5f/clinexpimmunol00193-0160-a.jpg

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ANTIBODY DEFICIENCY SYNDROME WITH INCREASED GAMMA1 M (B2 M) GLOBULINS AND ABSENT GAMMA1 A (B2 A) GLOBULINS AND GAMMA2 (G) GLOBULINS.
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