EXPRESSION OF AN X-LINKED GENE FAMILY (XLR) IN LATE-STAGE B-CELLS AND ITS ALTERATION BY THE XID MUTATION
EXPRESSION OF AN X-LINKED GENE FAMILY (XLR) IN LATE-STAGE B-CELLS AND ITS ALTERATION BY THE XID MUTATION
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DOI：
10.1038/314372a0
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发表时间：
1985-01-01
期刊：
影响因子：
64.8
通讯作者：
DAVIS, MM
中科院分区：
文献类型：
--
作者：
COHEN, DI;STEINBERG, AD;DAVIS, MM
研究最广泛的X-连锁免疫缺陷病之一是小鼠品系CBA/N的突变（综述见参考文献1）。这种突变可能涉及成熟缺陷，因为氧化动物无法产生针对可溶性多糖抗原的抗体，这一功能通常归因于晚期B细胞2，3。此外，使用单克隆抗体的研究已经确定了一种B细胞表面抗原（BLA-2或14 G8），其在大多数或所有未成熟B淋巴细胞上表达，但在成熟脾B淋巴细胞亚群上不表达4，5;这种晚期14 G8抗原阴性的脾B细胞亚群显然不存在于携带thexiddefect的小鼠中5。在随附的论文中，我们描述了一个cDNA克隆的分离，该克隆识别X染色体上的一个基因家族，其中至少有一些成员与该性状密切相关。我们在这里报告，这个基因家族，XLR，转录在某些B-和T-细胞系肿瘤，但不是在巨噬细胞肿瘤，或肝或肾细胞。我们发现它主要在晚期14 G8阴性B细胞肿瘤和浆细胞瘤中转录，但不在未成熟B细胞或前B细胞肿瘤中转录。我们能够在所有12个来自正常X染色体小鼠的浆细胞瘤（分泌性B细胞瘤）中检测到转录，但在3个携带该突变的浆细胞瘤中检测不到转录。这些数据，结合限制性片段长度多态性分析，将XLR基因家族与exidmutation 6联系起来，表明exiddefect发生在该基因家族的一个成员中。
One of the most extensively studied X-linked immunodeficiency disorder is thexidmutation of the mouse strain CBA/N (reviewed in ref. 1). This mutation may involve a maturational defect asxidanimals are unable to raise antibodies to soluble polysaccharide antigens, a function normally attributed to late-stage B cells2,3. Moreover, studies using monoclonal antibodies have defined a B-cell surface antigen (BLA-2 or 14G8) that is expressed on most or all immature B lymphocytes, but not on a subpopulation of mature splenic B lymphocytes4,5; this late-stage, 14G8 antigen-negative splenic B-cell subpopulation is apparently absent from mice bearing thexiddefect5. In the accompanying paper6we describe the isolation of a cDNA clone recognizing a family of genes on the X chromosome, at least some of whose members are closely linked to thexidtrait. We report here that this gene family, XLR, is transcribed in certain B- and T-cell lineage tumours, but not in macrophage tumours, or liver or kidney cells. We show that it is transcribed principally in late-stage, 14G8-negative B-cell tumours and plasmacytomas, but not in immature B-cell or pre-B-cell tumours. We are able to detect transcription in all of 12 plasmacytomas (secretory B-cell tumours) derived from mice with normal X chromosomes, but not in three plasmacytomas carrying thexidmutation. These data, combined with the restriction fragment length polymorphism analysis linking the XLR gene family to thexidmutation6, suggests that thexiddefect occurs within a member of this gene family.