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干燥综合征患者外周血B细胞中主要免疫球蛋白帽化缺陷

Major immunoglobulin capping deficiency in the peripheral blood B cells of patients with Sjögren's syndrome.

作者信息

Bataille R, Zhang X G, Duperray C, Rajzbaum G, Brochier J, Klein B

机构信息

INSERM U 291, Immunopathologie des Affections Tumorales et Autoimmunes, Montpellier, France.

出版信息

Immunology. 1992 Mar;75(3):550-2.

Abstract

The capping of surface immunoglobulins (sIg) is a major characteristic of normal B lymphocytes. Thus, we have investigated sIg capping by peripheral blood (PB) B cells of patients with Sjögren's syndrome (SS) and we have found a major deficiency in these patients. In 12 healthy donors (HD), 8 +/- 2.8% of PB mononuclear cells were B cells (i.e. expressing the B-cell antigens CD19, CD20 and CD21 simultaneously) and more than 90% of these PB B cells were able to cap their sIg. In 12 experiments performed using PB lymphocytes from seven patients with SS, a major capping deficiency was noted with only 30% of PB B lymphocytes being able to cap sIg. This defect was not related to an expansion of the B-cell subpopulation expressing the CD5 antigen and was not observed in five patients with rheumatoid arthritis lacking SS. Capping of sIg via antigen binding (i.e. antigenic modulation) constitutes the initial signal for B-cell activation. This process is involved in anti-viral defence and could have a potential pathogenetic role in autoimmune diseases. This impaired B-cell function presently described represents an immune defect which could be important in the pathogenesis of SS.

摘要

表面免疫球蛋白(sIg)的帽化是正常B淋巴细胞的一个主要特征。因此,我们研究了干燥综合征(SS)患者外周血(PB)B细胞的sIg帽化情况,发现这些患者存在严重缺陷。在12名健康供体(HD)中,8±2.8%的PB单核细胞为B细胞(即同时表达B细胞抗原CD19、CD20和CD21),其中超过90%的PB B细胞能够使其sIg形成帽状。在使用7例SS患者的PB淋巴细胞进行的12次实验中,发现存在严重的帽化缺陷,只有30%的PB B淋巴细胞能够使sIg形成帽状。这种缺陷与表达CD5抗原的B细胞亚群的扩增无关,在5例无SS的类风湿关节炎患者中未观察到。通过抗原结合使sIg形成帽状(即抗原调节)是B细胞活化的初始信号。这一过程参与抗病毒防御,可能在自身免疫性疾病中具有潜在的致病作用。目前所描述的这种受损的B细胞功能代表了一种免疫缺陷,可能在SS的发病机制中起重要作用。

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Sjögren's syndrome. Proposed criteria for classification.干燥综合征。分类的推荐标准。
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Rheumatoid factor secretion from human Leu-1+ B cells.人Leu-1⁺ B细胞分泌类风湿因子。
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