NF-κB and inflammation in genetic disease
NF-κB and inflammation in genetic disease
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DOI：
10.1016/j.bcp.2006.08.006
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发表时间：
2006-10-30
影响因子：
5.8
通讯作者：
Courtois, Gilles
中科院分区：
文献类型：
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作者：
Sebban, Helene;Courtois, Gilles
通过响应促炎细胞因子（例如 IL-1 beta 和 TNF-alpha）并控制自身多种炎症介质的表达，NF-κ B 在控制表征炎症过程的正确事件顺序方面发挥着关键作用。虽然过度的 NF-κ B 激活通常与许多不同组织的炎症症状相关，但 NF-κ B 激活受损也会产生炎症。患有色素失禁遗传病的人类就属于这种情况，并表现出严重的皮肤炎症。确定了这种病理学的分子基础，即影响 NEMO 基因编码的突变，使得能够生产用于研究该疾病的小鼠模型。他们的表征支持这样的观点：体内需要对 NF-kappa B 信号通路进行非常严格的正向和负向调节，以确保不仅对损伤或感染有微调反应，而且还能维持组织稳态。 (c) 2006 Elsevier Inc. 保留所有权利。
By responding to pro-inflammatory cytokines, such as IL-1 beta and TNF-alpha, and controlling itself the expression of numerous mediators of inflammation, NF-kappa B plays a pivotal role in controlling the proper sequence of events characterizing the inflammation process. Although excessive NF-kappa B activation is often associated with inflammatory signs in many different tissues, impaired NF-kappa B activation can also generate inflammation. This is the case in humans suffering from the genetic disease incontinentia pigmenti that exhibit severe skin inflammation. Identifying the molecular basis of this pathology, mutations affecting the gene coding for NEMO, has allowed production of mouse models for investigating the disease. Their characterization supports the view that a very tight positive and negative regulation of the NF-kappa B signaling pathway is required in vivo to ensure not only a fine-tuned response to injury or infection but also to maintain tissue homeostasis. (c) 2006 Elsevier Inc. All rights reserved.