A NEW MUTATION, ALY, THAT INDUCES A GENERALIZED LACK OF LYMPH-NODES ACCOMPANIED BY IMMUNODEFICIENCY IN MICE

A NEW MUTATION, ALY, THAT INDUCES A GENERALIZED LACK OF LYMPH-NODES ACCOMPANIED BY IMMUNODEFICIENCY IN MICE
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DOI:
10.1002/eji.1830240224
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发表时间:
1994-02-01
影响因子:
5.4
通讯作者:
SHIBATA, Y
SHIBATA, Y
中科院分区:
医学3区
文献类型:
--
作者:
MIYAWAKI, S;NAKAMURA, K;SHIBATA, Y

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我们在小鼠体内发现了一种新的自发性常染色体隐性突变,该突变会导致全身性淋巴结和派尔氏淋巴结缺失。该突变体被命名为“alymphoplasia”,基因符号为“aly”。 aly/aly 小鼠的脾脏缺乏明确的淋巴滤泡,并且胸腺没有显示出明显的皮质髓质区别。突变纯合子缺乏体液和细胞介导的免疫功能,并且非常容易受到感染。他们的血清中 IgM 水平降低,IgG 和 IgA 水平严重降低,并且不会排斥同种异体皮肤移植物。然而,根据细胞表面抗原确定,它们具有成熟的 T 细胞和 B 细胞。骨髓移植实验的结果表明,间充质疾病可能是 aly 小鼠缺乏淋巴结和免疫缺陷的原因。 aly 突变小鼠可能是一种有用的原发性免疫缺陷动物模型,nu(裸)和 scid(严重联合免疫缺陷)小鼠也是如此。
We have found a new spontaneous autosomal recessive mutation in mice that causes a systemic absence of lymph nodes and Peyer's patches. The name ''alymphoplasia'', with the gene symbol ''aly'', is proposed for this mutant. The spleen of aly/aly mice is devoid of well-defined lymphoid follicles, and the thymus does not show a clear cortical-medullary distinction. The mutant homozygotes are deficient in both humoral and cell-mediated immune functions, and are highly susceptible to infections. They have a reduced level of IgM and severely depressed levels of IgG and IgA in their sera, and do not reject allogeneic skin grafts. However, they have mature T and B cells as determined from their cell surface antigens. The results of bone marrow transplantation experiments suggest a mesenchymal disorder as a possible cause of the lack of lymph nodes and of immunodeficiency in the aly mouse. The aly mutant mouse may be a useful animal model of primary immunodeficiency, as are the nu (nude) and scid (severe combined immunodeficiency) mice.