Int. J. Mol. Sci. 2017, 18(2), 382; doi:10.3390/ijms18020382
Endoplasmic Reticulum (ER) Stress and Endocrine Disorders
1
Division of Developmental Genetics, Institute of Resource Development and Analysis, Kumamoto University, Kumamoto 860-0811, Japan
2
Department of Biochemistry and Molecular Biology, Graduate School of Life Science, University of Hyogo, Hyogo 678-1297, Japan
3
Division of Endocrinology and Metabolism, Tokyo Metropolitan Children’s Medical Center, Tokyo 183-8561, Japan
*
Authors to whom correspondence should be addressed.
Academic Editor: Masato Matsuoka
Received: 22 December 2016 / Revised: 24 January 2017 / Accepted: 3 February 2017 / Published: 11 February 2017
(This article belongs to the Special Issue Modulators of Endoplasmic Reticulum Stress 2016)
Abstract
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. View Full-TextKeywords:
endoplasmic reticulum stress; endocrine disorder; PKR-like endoplasmic reticulum kinase (PERK); activating transcription factor 6 (ATF6); inositol requirement 1 (IRE1); old astrocyte specifically induced substance (OASIS) family; chemical chaperone
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).
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Ariyasu, D.; Yoshida, H.; Hasegawa, Y. Endoplasmic Reticulum (ER) Stress and Endocrine Disorders. Int. J. Mol. Sci. 2017, 18, 382.
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