Yasuda N, Kanoh T, Uchino H
Clin Exp Immunol. 1981 Oct;46(1):142-8.
J chain synthesis was investigated by in vitro pokeweed mitogen (PWM) stimulated peripheral blood lymphocyte (PBL) cultures in eight patients with selective IgA deficiency and compared with that of normal persons. In normals, all IgM-containing cells always had the J chain but only in a portion of IgG- and IgA-containing cells was J chain detectable. The percentage of J chain-positive cells amongst IgG or IgA cells increased during culture, reached a peak at days 5-6 or 6-7, respectively, and then decreased. IgA-deficient patients had very few IgA-containing cells and an increased number and percentage of J chain-positive IgG cells, except for one patient, who had a significant number of IgA-containing cells without IgA secretion into the culture supernatants. Measurement of Ig in culture supernatants by radioimmunoassay revealed that lymphocytes from seven patients secreted significantly smaller amounts of IgG and IgM than did the normal controls, in addition to the defect in IgA production. These results suggested the presence of some ontogenetic relationship between J chain-positive IgG cells and the precursors of IgA-producing cells, and some functional immaturity of most IgG-producing clones seen in patients with selective IgA deficiency.
通过体外美洲商陆丝裂原(PWM)刺激的外周血淋巴细胞(PBL)培养,研究了8例选择性IgA缺乏患者的J链合成情况,并与正常人进行了比较。在正常人中,所有含IgM的细胞均有J链,但仅在一部分含IgG和IgA的细胞中可检测到J链。培养过程中,IgG或IgA细胞中J链阳性细胞的百分比增加,分别在第5 - 6天或6 - 7天达到峰值,然后下降。除1例患者外,IgA缺乏患者含IgA的细胞极少,J链阳性IgG细胞的数量和百分比增加,该例患者有大量含IgA的细胞,但无IgA分泌到培养上清液中。通过放射免疫测定培养上清液中的Ig发现,除了IgA产生缺陷外,7例患者的淋巴细胞分泌的IgG和IgM明显少于正常对照。这些结果提示J链阳性IgG细胞与IgA产生细胞的前体之间存在某种个体发生学关系,以及选择性IgA缺乏患者中大多数产生IgG的克隆存在某种功能不成熟。