INTERLEUKIN-2 RECEPTOR GAMMA-CHAIN - A FUNCTIONAL COMPONENT OF THE INTERLEUKIN-7 RECEPTOR
INTERLEUKIN-2 RECEPTOR GAMMA-CHAIN - A FUNCTIONAL COMPONENT OF THE INTERLEUKIN-7 RECEPTOR
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DOI：
10.1126/science.8266077
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发表时间：
1993-12-17
期刊：
影响因子：
56.9
通讯作者：
LEONARD, WJ
中科院分区：
文献类型：
--
作者：
NOGUCHI, M;NAKAMURA, Y;LEONARD, WJ
白细胞介素-2受体γ链（IL-2 R γ）是功能性IL-2受体的必要组分。IL-2 R γ突变导致人类X连锁严重联合免疫缺陷（XSCID），这是一种以存在很少或没有T细胞为特征的疾病。相比之下，IL-2缺乏的SCID患者和IL-2缺乏的小鼠具有正常数量的T细胞，这表明IL-2 R γ是一种以上细胞因子受体的一部分。通过使用化学交联，显示IL-2 R γ与IL-7受体物理相关。IL-2 R γ的存在增强了IL-7结合亲和力和IL-7内化的效率。这些发现可能有助于解释XSCID的缺陷。鉴于其在一种以上细胞因子受体系统中的作用，建议将共同γ链（γ（c））命名为IL-2 R γ。
The interleukin-2 receptor gamma chain (IL-2Rgamma) is a necessary component of functional IL-2 receptors. IL-2Rgamma mutations result in X-linked severe combined immunodeficiency (XSCID) in humans, a disease characterized by the presence of few or no T cells. In contrast, SCID patients with IL-2 deficiency and IL-2-deficient mice have normal numbers of T cells, suggesting that IL-2Rgamma is part of more than one cytokine receptor. By using chemical cross-linking, IL-2Rgamma was shown to be physically associated with the IL-7 receptor. The presence of IL-2Rgamma augmented both IL-7 binding affinity and the efficiency of internalization of IL-7. These findings may help explain the defects of XSCID. Given its role in more than one cytokine receptor system, the common gamma chain (gamma(c) is proposed as the designation for IL-2Rgamma.