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严重低丙种球蛋白血症患者外周血淋巴细胞及培养的淋巴母细胞产生和分泌免疫球蛋白的情况

Biosynthesis and secretion of immunoglobulins by peripheral blood lymphocytes in severe hypogammaglobulinemia and by cultured lymphoblast cells.

作者信息

Choi Y S, Good R A

出版信息

Birth Defects Orig Artic Ser. 1975;11(1):81-6.

PMID:1170905
Abstract

Peripheral blood lymphocytes of two patients with infantile X-linked agammaglobulinemia could neither synthesize nor secrete immunoglobulins in detectable amounts. Peripheral blood lymphocytes of patients with common variable type of hypogammaglobulinemia synthesize but do not secrete immunoglobulins. Serologic precipitation with antiimmunoglobulin serum and acrylamide-gel analysis of immunoglobulins synthesized by lymphocytes of these patients showed predominant mu-chain, as compared to immunoglobulins synthesized by normal lymphocytes. Biosynthesis and secretion of immunoglobulins by cultured lymphoblast cells from normal persons was very much analogous to that of lymphocytes of hypogammaglobulinemic patients. These findings suggest that lymphocytes of patients with common variable type of hypogammaglobulinemia are immature B cells like cultured lymphoblast cells, and that a block in cytodifferentiation of IgM to IgG-producing cells is present in their B cells.

摘要

两名患有婴儿X连锁无丙种球蛋白血症的患者的外周血淋巴细胞既不能合成也不能分泌可检测量的免疫球蛋白。常见变异型低丙种球蛋白血症患者的外周血淋巴细胞能合成但不能分泌免疫球蛋白。用抗免疫球蛋白血清进行的血清学沉淀以及对这些患者淋巴细胞合成的免疫球蛋白进行的丙烯酰胺凝胶分析表明,与正常淋巴细胞合成的免疫球蛋白相比,其主要为μ链。正常人培养的淋巴母细胞对免疫球蛋白的生物合成和分泌与低丙种球蛋白血症患者的淋巴细胞非常相似。这些发现表明,常见变异型低丙种球蛋白血症患者的淋巴细胞是未成熟的B细胞,类似于培养的淋巴母细胞,并且其B细胞中存在从产生IgM的细胞向产生IgG的细胞的细胞分化阻滞。

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