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首页> 外文期刊>Clinical and diagnostic laboratory immunology >Identification of a Subset of Common Variable Immunodeficiency Patients with Impaired B-Cell Protein Tyrosine Phosphorylation
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Identification of a Subset of Common Variable Immunodeficiency Patients with Impaired B-Cell Protein Tyrosine Phosphorylation

机译：B细胞蛋白酪氨酸磷酸化受损的常见可变免疫缺陷病患者亚群的鉴定

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摘要

The mechanisms responsible for common variable immunodeficiency syndrome (CVID) are as yet unknown. In the present study, we show that the B-cell dysfunction in a subset of CVID patients is caused by defective protein tyrosine phosphorylation (PTP). We demonstrated that the PTP level and immunoglobulin (Ig) secretion malfunctions can be successfully repaired when normal plasma membrane components are implanted into these patients’ B cells. Stimulation of CVID patients’ peripheral blood mononucleated cells with anti-Ig antibody revealed that 7 of 11 patients had lower PTP levels than those found in the normal donor cells. Plasma membrane implantation to the cells of these patients resulted in elevated PTP levels which reached normal levels upon stimulation with anti-human Ig antibody. The results revealed two distinct groups of CVID patients. The first group included patients whose B cells expressed low PTP levels after Ig stimulation. In these patients the plasma membrane implantation restored the normal PTP level as well as the ability to secrete IgM and/or IgG after B-cell stimulation. In the second group, patients whose B cells expressed a normal PTP level after Ig stimulation, with no restoration of their ability to secrete Ig upon plasma membrane implantation and lipopolysaccharide stimulation. We conclude that the first group has an early signal transduction defect located in the B-cell plasma membrane, while in the second group the defect is located elsewhere.
机译：尚不清楚导致常见的可变免疫缺陷综合症（CVID）的机制。在本研究中，我们显示了一部分CVID患者的B细胞功能障碍是由蛋白质酪氨酸磷酸化缺陷（PTP）引起的。我们证明，将正常的质膜成分植入这些患者的B细胞后，可以成功修复PTP水平和免疫球蛋白（Ig）分泌异常。用抗Ig抗体刺激CVID患者的外周血单核细胞后，发现11名患者中有7名的PTP水平低于正常供体细胞中的PTP水平。这些患者细胞的质膜植入导致PTP水平升高，在用抗人Ig抗体刺激后达到正常水平。结果揭示了两组不同的CVID患者。第一组包括Ig刺激后B细胞表达低PTP水平的患者。在这些患者中，质膜植入恢复了正常的PTP水平以及B细胞刺激后分泌IgM和/或IgG的能力。在第二组中，B细胞在Ig刺激后表达正常PTP水平的患者，在质膜植入和脂多糖刺激后其分泌Ig的能力没有恢复。我们得出的结论是，第一组的早期信号传导缺陷位于B细胞质膜上，而第二组的缺陷位于其他位置。

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