Lebreton J P, Fontaine M, Rousseaux J, Youinou P, Hurez D, Rivat-Peran L, Bernards J P
Clin Exp Immunol. 1982 Jan;47(1):206-16.
The serum of a female patient studied over 7 months initially showed, in addition to normal residual IgG, two abnormal IgG proteins and in the last 3 months of the disease showed only one abnormal IgG protein. Gm typing and serological subclass determinations revealed an imbalance of allelic forms within the IgG1 subclass during the disease. The IgG2 level remained markedly elevated throughout the study. The two abnormal IgG-related proteins were devoid of light chains. The abnormal gamma cathodic immunoglobulin and the abnormal beta 2-immunoglobulin were Fc-like and covalently disulphide linked, with molecular weights of 60,000 daltons (N terminal, SER) and 72,000 daltons (N terminal, GLY) respectively. The first belonged to the IgG1 subclass and the second to the IgG2 subclass. No abnormal proteolytic activity was noted and plasma cells reacted with anti-gamma-chain antisera only. We hypothesize that the molecular defect leading to the deleted chains was an early event, preceding the differentiation into plasma cells which produced the two IgG1 and IgG2 deleted H chains.
对一名女性患者进行了7个月的研究，其血清最初除正常残留IgG外，还显示出两种异常IgG蛋白，而在疾病的最后3个月仅显示一种异常IgG蛋白。Gm分型和血清学亚类测定显示，疾病期间IgG1亚类内等位基因形式失衡。在整个研究过程中，IgG2水平显著升高。两种异常的IgG相关蛋白均无轻链。异常的γ阴极免疫球蛋白和异常的β2免疫球蛋白类似Fc，通过共价二硫键连接，分子量分别为60,000道尔顿（N端，SER）和72,000道尔顿（N端，GLY）。第一种属于IgG1亚类，第二种属于IgG2亚类。未观察到异常的蛋白水解活性，浆细胞仅与抗γ链抗血清发生反应。我们推测，导致缺失链的分子缺陷是一个早期事件，发生在分化为产生两条IgG1和IgG2缺失重链的浆细胞之前。
