Noguchi M, Nakamura Y, Russell S M, Ziegler S F, Tsang M, Cao X, Leonard W J
Section on Pulmonary and Molecular Immunology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892.
Science. 1993 Dec 17;262(5141):1877-80. doi: 10.1126/science.8266077.
The interleukin-2 receptor gamma chain (IL-2R gamma) is a necessary component of functional IL-2 receptors. IL-2R gamma 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-2R gamma is part of more than one cytokine receptor. By using chemical cross-linking, IL-2R gamma was shown to be physically associated with the IL-7 receptor. The presence of IL-2R gamma 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-2R gamma.
白细胞介素-2受体γ链(IL-2Rγ)是功能性IL-2受体的必需组成部分。IL-2Rγ突变会导致人类X连锁重症联合免疫缺陷病(XSCID),该病的特征是T细胞数量很少或没有。相比之下,IL-2缺乏的重症联合免疫缺陷病患者和IL-2缺乏的小鼠T细胞数量正常,这表明IL-2Rγ是多种细胞因子受体的一部分。通过化学交联,发现IL-2Rγ与IL-7受体存在物理关联。IL-2Rγ的存在增强了IL-7的结合亲和力以及IL-7的内化效率。这些发现可能有助于解释XSCID的缺陷。鉴于其在多种细胞因子受体系统中的作用,共同γ链(γc)被提议作为IL-2Rγ的命名。