Kondo N, Inoue R, Kasahara K, Fukao T, Kaneko H, Tashita H, Teramoto T
Department of Pediatrics, Gifu University School of Medicine, Japan.
Scand J Immunol. 1997 Feb;45(2):227-30. doi: 10.1046/j.1365-3083.1997.d01-387.x.
The specific defect that causes IgG2 deficiency, which is one of the primary immunodeficiencies, is unknown. Recently, it was shown that interferon-gamma (IFN-gamma) induces synthesis of human germline C gamma 2 transcripts. In the authors' previous study and the present one, peripheral blood lymphocytes (PBLs) of all five tested patients with IgG2 deficiency failed to produce enough IFN-gamma when stimulated with phytohaemagglutinin or concanavalin A although they produced a sufficient amount of interleukin-2 (IL-2). The low level of IgG2 production in pokeweed mitogen-stimulated PBLs of four tested patients was improved by the addition of recombinant IFN-gamma. In this study, the amount of IFN-gamma messenger RNA showed various degrees of reduction in all five tested patients. Sequence analysis of the IFN-gamma coding regions and flanking regions revealed neither a point mutation nor a deletion for any of the patients. Thus the results suggest that the reduced expression of IFN-gamma messenger RNA may play an important role in the IgG2 deficiency of these patients.
导致原发性免疫缺陷之一的IgG2缺乏症的具体缺陷尚不清楚。最近的研究表明,γ干扰素(IFN-γ)可诱导人胚系Cγ2转录本的合成。在作者之前的研究以及本研究中,尽管五名接受检测的IgG2缺乏症患者的外周血淋巴细胞(PBL)在受到植物血凝素或伴刀豆球蛋白A刺激时能产生足够量的白细胞介素-2(IL-2),但均无法产生足够的IFN-γ。在四名接受检测的患者中,通过添加重组IFN-γ,商陆有丝分裂原刺激的PBL中IgG2的低产量得到了改善。在本研究中,所有五名接受检测的患者中IFN-γ信使核糖核酸的量均呈现出不同程度的减少。对IFN-γ编码区和侧翼区的序列分析显示,所有患者均未出现点突变或缺失。因此,结果表明IFN-γ信使核糖核酸表达的降低可能在这些患者的IgG2缺乏症中起重要作用。