RELATIVE INCREASE OF T-CELLS EXPRESSING THE GAMMA-DELTA RATHER THAN THE ALPHA-BETA RECEPTOR IN ATAXIA TELANGIECTASIA
RELATIVE INCREASE OF T-CELLS EXPRESSING THE GAMMA-DELTA RATHER THAN THE ALPHA-BETA RECEPTOR IN ATAXIA TELANGIECTASIA
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DOI：
10.1056/nejm199001113220201
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发表时间：
1990-01-11
影响因子：
158.5
通讯作者：
FIORILLI, M
中科院分区：
文献类型：
--
作者：
CARBONARI, M;CHERCHI, M;FIORILLI, M
在共济失调-毛细血管扩张症中，B细胞和T细胞缺陷被认为是由于免疫球蛋白和T细胞受体基因重排的缺陷。T细胞通过两种类型的CD 3相关受体识别抗原：α-CD 3受体。β的成熟细胞上的γ链和γ/.δ的主要是在未成熟的细胞上。我们研究了10名患有共济失调-毛细血管扩张症的患者，发现大多数患者具有相对增加的携带γ/β的循环T细胞。δ的受体而不是α/。β的与正常对照组比较，P < 0.001。具有其他免疫缺陷的患者，包括8名具有普通可变免疫缺陷的患者、1名具有Wiskott-Aldrich综合征的患者、2名具有高免疫球蛋白血症E综合征的患者和1名具有严重联合免疫缺陷的患者，具有正常的γ/γ比值。三角洲-方位为α/。贝塔产生细胞。γ/的显著优势。三角洲-在一个原发性T细胞缺陷的患者中发现了携带T细胞的细胞。γ/.的相对增加三角洲-共济失调-毛细血管扩张症患者中携带T细胞的细胞主要是与单克隆抗体BB 3反应的细胞，BB 3是选择性表达T细胞受体的C γ 1基因产物的T细胞的明显不同的亚群。尽管它们具有正常的γ/γ比值。三角洲-方位为α/。β-的携带T细胞的普通可变免疫缺陷患者与单克隆抗体δ-1反应的表达C γ 2的T细胞数量显著增加（p = 0.01）。TCS-1我们得出结论，γ/.三角洲-方位为α/。贝塔在共济失调-毛细血管扩张症中携带T细胞可能反映了干扰T细胞和B细胞基因重排的重组缺陷和不能修复DNA损伤。
In ataxia-telangiectasia, B-cell and T-cell deficiencies are thought to be due to a defect of rearrangements of immunoglobulin and T-cell receptor genes. T cell recognize antigens through two types of CD3-associated receptors: .alpha.-.beta. chains on mature cells and .gamma./.delta. chains mostly on immature cells. We studied 10 patients with ataxia-telangiectasia and found that most had a relative increase of circulating T cells bearing .gamma./.delta. receptors rather then .alpha./.beta. receptors, as compared with normal subjects (P < 0.001). Patients with other immune deficits, including eight with common variable immunodeficiency, one with Wiskott-Aldrich syndrome, two with hyperimmunoglobulinemia E syndrome, and one with severe combined immunodeficiency, had normal ratios of .gamma./.delta.-bearing to .alpha./.beta.-bearing cells. A marked predominance of .gamma./.delta.-bearing T cells was found in a patient with a primary T-cell defect. The relative increase in .gamma./.delta.-bearing T cells in the patients with ataxia-telangiectasia was largely accounted for by cells that reacted with the monoclonal antibody BB3, an apparently distinct subset of T cells that selectively express the C .gamma.1 gene product of the T-cell receptor. Although they had normal ratios of .gamma./.delta.-bearing to .alpha./.beta.-bearing T cells, the patients with common variable immunodeficiency had a significant increase (p = 0.01) in the number of T cells expressing C .gamma.2 that reacted with the monoclonal antibody .delta.-TCS-1. We conclude that the increased ratio of .gamma./.delta.-bearing to .alpha./.beta.-bearing T cells in ataxia-telangiectasia may reflect both a recombinational defect that interferes with T-cell and B-cell gene rearrangements and an inability to repair damage to the DNA.