Recent Insights into the Role of Unfolded Protein Response in ER Stress in Health and Disease.
Recent Insights into the Role of Unfolded Protein Response in ER Stress in Health and Disease.
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DOI：
10.3389/fcell.2017.00048
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发表时间：
2017
影响因子：
5.5
通讯作者：
Kõks S
中科院分区：
文献类型：
--
作者：
Lindholm D;Korhonen L;Eriksson O;Kõks S
未折叠应激反应（UPR）是一种保守的细胞途径，参与蛋白质质量控制，以在以细胞应激为特征的不同条件和疾病状态下维持稳态。虽然三个一般方案和基因诱导的UPR是相当完善的，开放的问题仍然包括UPR在人类疾病中的确切作用和细胞应激信号传导过程中不同的传感器系统之间的相互作用。特别是，正常适应性和促生存的UPR途径如何变成导致持续内质网（ER）应激和细胞死亡的有害过程的问题需要更多的研究。普遍定期审议也被命名为一个朋友，具有多重人格，我们需要更好地了解，以充分认识其在正常生理和疾病病理中的作用。UPR与其他细胞器（包括线粒体）以及细胞应激信号和降解途径（如自噬和泛素蛋白酶体系统）相互作用。在这里，我们回顾了目前的概念和机制的UPR研究在不同的细胞和模型系统，并强调UPR和相关的压力信号在各种人类疾病的相关性。
Unfolded stress response (UPR) is a conserved cellular pathway involved in protein quality control to maintain homeostasis under different conditions and disease states characterized by cell stress. Although three general schemes of and genes induced by UPR are rather well-established, open questions remain including the precise role of UPR in human diseases and the interactions between different sensor systems during cell stress signaling. Particularly, the issue how the normally adaptive and pro-survival UPR pathway turns into a deleterious process causing sustained endoplasmic reticulum (ER) stress and cell death requires more studies. UPR is also named a friend with multiple personalities that we need to understand better to fully recognize its role in normal physiology and in disease pathology. UPR interacts with other organelles including mitochondria, and with cell stress signals and degradation pathways such as autophagy and the ubiquitin proteasome system. Here we review current concepts and mechanisms of UPR as studied in different cells and model systems and highlight the relevance of UPR and related stress signals in various human diseases.