Endoplasmic Reticulum (ER) Stress and Endocrine Disorders.
Endoplasmic Reticulum (ER) Stress and Endocrine Disorders.
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DOI：
10.3390/ijms18020382
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发表时间：
2017-02-11
影响因子：
5.6
通讯作者：
Hasegawa Y
中科院分区：
文献类型：
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作者：
Ariyasu D;Yoshida H;Hasegawa Y
内质网是合成和折叠分泌蛋白和膜蛋白的细胞器。保留在ER内的未折叠蛋白质可引起ER应激。真核细胞有一种称为“未折叠蛋白反应”（UPR）的防御系统，可以保护细胞免受ER应激。当ER应激超过UPR的能力时，细胞发生凋亡，这已被揭示会导致人类疾病。虽然神经退行性疾病是众所周知的ER应激相关疾病，但已发现内分泌疾病也与ER应激有关。在这篇综述中，我们重点关注ER应激相关的人类内分泌疾病。除了糖尿病，这是很好的特点，几个相对罕见的遗传性疾病，如家族性神经垂体尿崩症（FNDI），Wolfram综合征，和孤立性生长激素缺乏症II型（IGHD2）在本文中进行了讨论。
The endoplasmic reticulum (ER) is the organelle where secretory and membrane proteins are synthesized and folded. Unfolded proteins that are retained within the ER can cause ER stress. Eukaryotic cells have a defense system called the “unfolded protein response” (UPR), which protects cells from ER stress. Cells undergo apoptosis when ER stress exceeds the capacity of the UPR, which has been revealed to cause human diseases. Although neurodegenerative diseases are well-known ER stress-related diseases, it has been discovered that endocrine diseases are also related to ER stress. In this review, we focus on ER stress-related human endocrine disorders. In addition to diabetes mellitus, which is well characterized, several relatively rare genetic disorders such as familial neurohypophyseal diabetes insipidus (FNDI), Wolfram syndrome, and isolated growth hormone deficiency type II (IGHD2) are discussed in this article.