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Etiology of congenital agammaglobulinemia: Growth defect of precursor B lymphoblastoid cell lines and immunoglobulin gene rearrangements.

先天性无丙种球蛋白血症的病因：前体 B 淋巴母细胞系的生长缺陷和免疫球蛋白基因重排。

基本信息

批准号：
62480224
负责人：
TSUCHIYA Shigeru
金额：
$ 3.97万
依托单位：
TOHOKU UNIVERSITY
依托单位国家：
日本
项目类别：
Grant-in-Aid for General Scientific Research (B)
财政年份：
1987
资助国家：
日本
起止时间：
1987 至 1988
项目状态：
已结题

项目摘要

Epstein-Barr virus (EBV) induced B lymphoblastoid cell lines (B-LCL) were established from bone marrow cells of the patients with congenital agammaglobulinemia (CAG).We found several immature B-LCL, some of which possess no cell-surface and cytoplastic immunoglobulins, or possess only cytoplasmic heavy chains. We examined the mode of expression of B-cell associated antigens on EBV-induced B-LCL and failed to detect any CAG specific and developmental stage-specific expression of known B-cell related antigens on those B-LCL. These facts indicate that expression of B cell associated antigens on EBV-induced B-LCL does not depend on the developmental stage of B cells, rather depend on the state of EBV infections.If we can detect LCL which express immunoglobulins with very unusual manner, those LCL will probably give us oppotunities to elucidate the etiology of CAG. Fortunately we found 2 such cell lines, K5 and K4, from the same CAG patient. K5 was very unique because they expressed only IgD (delea,lambda) on the surface and produced IgD. K4 was also very unique because they produced lambda heavy chain, and both kappa and lambda light chains. Cell cloning experiments repeated 3 times definitely confirmed that K4 cells produced both kappa and lambda light chains with single mu chains. The pattern of immunoglobolin production seen on K4 and K5 was probably the first ones in the literature. We are going to clone immunoglobuline genes of K4 and K5, and to elucidate the genetic mechanism of the expression of unusual immunoglobulins.

用eb病毒（EBV）诱导的先天性无球蛋白血症（CAG）患者骨髓细胞建立B淋巴母细胞样细胞株（B- lcl）。我们发现了几种未成熟的B-LCL，其中一些不具有细胞表面和细胞质免疫球蛋白，或仅具有细胞质重链。我们检测了b细胞相关抗原在ebv诱导的B-LCL中的表达模式，但未能检测到任何CAG特异性和发育阶段特异性的已知b细胞相关抗原在这些B-LCL中的表达。这些事实表明，在EBV诱导的B- lcl中，B细胞相关抗原的表达并不取决于B细胞的发育阶段，而是取决于EBV感染的状态。如果我们能检测到表达免疫球蛋白方式异常的小细胞淋巴瘤，这些小细胞淋巴瘤可能会为我们阐明CAG的病因提供机会。幸运的是，我们从同一CAG患者身上发现了2个这样的细胞系，K5和K4。K5非常独特，因为它们只在表面表达IgD （delea,lambda）并产生IgD。K4也非常独特，因为它们产生了重链，以及轻链和重链。重复3次的细胞克隆实验明确地证实了K4细胞产生kappa和lambda轻链，且有单个mu链。在K4和K5上观察到的免疫球蛋白生成模式可能是文献中最早发现的。我们将克隆免疫球蛋白基因K4和K5，并阐明异常免疫球蛋白表达的遗传机制。