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人类遗传性细胞因子和细胞因子受体缺陷

Inherited cytokine and cytokine receptor deficiencies in man.

作者信息

Di Santo J P

机构信息

INSERM U429, Hôpital Necker-Enfants Malades, Paris, France.

出版信息

Int Rev Immunol. 1998;17(1-4):103-20. doi: 10.3109/08830189809084489.

Abstract

Over the past decades, cytokines and their receptors have been shown to play a decisive role in the differentiation of both innate and adaptive immunity. The essential roles of cytokine/receptor pathways in vivo, however, have remained elusive and poorly defined. In some cases, primary immunodeficiency syndromes have provided the natural models in which the role of cytokines and their receptors in the development and function of the immune system have been elucidated. Animal models of cytokine/receptor deficiencies generated through gene targeting have also played a decisive factor in identifying the true biological roles of cytokine/receptor pathways. The end result of these approaches has been an enormous advance in our understanding of the cytokine control of normal and pathological human conditions, as well as the advent of new diagnostic tools and novel therapies.

摘要

在过去几十年中,细胞因子及其受体已被证明在先天性免疫和适应性免疫的分化中起决定性作用。然而,细胞因子/受体通路在体内的重要作用仍不清楚且定义不明确。在某些情况下,原发性免疫缺陷综合征提供了天然模型,其中细胞因子及其受体在免疫系统发育和功能中的作用已得到阐明。通过基因靶向产生的细胞因子/受体缺陷动物模型在确定细胞因子/受体通路的真正生物学作用方面也发挥了决定性作用。这些方法的最终结果是,我们对细胞因子对正常和病理人类状况的控制有了巨大进展,同时也出现了新的诊断工具和新疗法。

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