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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Expansion of clonotype-restricted HLA-identical maternal CD4(+) T cells in a patient with severe combined immunodeficiency and a homozygous mutation in the Artemis gene.
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Expansion of clonotype-restricted HLA-identical maternal CD4(+) T cells in a patient with severe combined immunodeficiency and a homozygous mutation in the Artemis gene.

机译：具有严重联合免疫缺陷和Artemis基因纯合突变的患者中克隆型限制的HLA相同的孕妇CD4（+）T细胞的扩增。

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摘要

We have observed a male infant with severe combined immunodeficiency (SCID) responsible for Artemis gene mutation, in whom marked expansion of the transplacentally grafted maternal CD4(+) T cells was observed in various tissues. His class I and II major histocompatibility antigens (MHC) were identical to his mother's. We analyzed the T-cell populations within target tissues at a molecular level in order to determine whether different T-cell clonotypes are expanded in different types of tissue. Prior to T-cell expansion, the T-cell receptor variable beta (TCRBV) 5.1 subfamily predominated in peripheral blood (PB) lymphocytes. Third complementarity determining region (CDR3) size spectratyping and amino acid sequencing showed that the range of T-cell clonotypes was very restricted. After T-cell expansion, different TCRBV subfamilies were found to predominate in different target tissues; these included TCRBV 5.1 and 17 in the PB, TCRBV 13 and 21.3 in the bone marrow, and TCRBV 17 in lymph nodes. CDR3 size analysis showed that the expression of different proliferating T-cell clonotypes remained restricted after T-cell expansion. These results indicate that highly restricted maternal T-cell clonotypes can markedly expand, possibly in response to tissue-specific antigens, in a MHC-identical recipient.
机译：我们已经观察到负责Artemis基因突变的严重合并免疫缺陷症（SCID）的男婴，其中在各个组织中观察到经胎盘移植的母体CD4（+）T细胞明显扩增。他的I类和II类主要组织相容性抗原（MHC）与母亲的相同。我们在分子水平上分析了靶组织内的T细胞群体，以确定是否在不同类型的组织中扩展了不同的T细胞克隆型。在T细胞扩增之前，T细胞受体可变β（TCRBV）5.1亚家族在外周血（PB）淋巴细胞中占主导地位。第三互补决定区（CDR3）的大小分型和氨基酸测序表明，T细胞克隆型的范围非常有限。 T细胞扩增后，在不同的靶组织中发现了不同的TCRBV亚家族。这些包括PB中的TCRBV 5.1和17，骨髓中的TCRBV 13和21.3以及淋巴结中的TCRBV 17。 CDR3大小分析表明，不同的增殖性T细胞克隆型的表达在T细胞扩增后仍然受到限制。这些结果表明，受高度限制的母亲T细胞克隆型可以在与MHC相同的受体中显着扩增，可能响应组织特异性抗原。

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