The impact of the unfolded protein response on human disease.

The impact of the unfolded protein response on human disease.
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DOI:
10.1083/jcb.201110131
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发表时间:
2012-06-25
期刊:
The Journal of cell biology
影响因子:
--
通讯作者:
Kaufman RJ
Kaufman RJ
中科院分区:
其他
文献类型:
--
作者:
Wang S;Kaufman RJ

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内质网(ER)的中心功能是协调细胞中蛋白质的生物合成和分泌活动。ER稳态的改变导致错误折叠/未折叠蛋白质在ER中的积累。为了维持ER稳态,真核细胞已经进化出未折叠蛋白反应(UPR),这是一种响应代谢、氧化应激和炎症反应途径的必需的适应性细胞内信号传导途径。UPR与多种疾病有关,包括代谢性疾病、神经退行性疾病、炎症性疾病和癌症。普遍定期审议的信号成分正在成为干预和治疗人类疾病的潜在靶点。
A central function of the endoplasmic reticulum (ER) is to coordinate protein biosynthetic and secretory activities in the cell. Alterations in ER homeostasis cause accumulation of misfolded/unfolded proteins in the ER. To maintain ER homeostasis, eukaryotic cells have evolved the unfolded protein response (UPR), an essential adaptive intracellular signaling pathway that responds to metabolic, oxidative stress, and inflammatory response pathways. The UPR has been implicated in a variety of diseases including metabolic disease, neurodegenerative disease, inflammatory disease, and cancer. Signaling components of the UPR are emerging as potential targets for intervention and treatment of human disease.
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