The unfolded protein response: its roles in physiological function and disease
- VernacularTitle:未折叠蛋白反应及其在生理功能与疾病中的作用
- Author:
Chen ZHI-XIN
1
;
Gu ZHEN-LUN
;
Qin ZHENG-HONG
Author Information
1. Soochow University School of Medicine
- Keywords:
unfolded protein response;
endoplasmic reticulum;
protein metabolism;
misfolded protein disease;
neurodegenerative diseases
- From:
Neuroscience Bulletin
2005;21(5):356-363
- CountryChina
- Language:Chinese
-
Abstract:
Cells respond to the accumulation of unfolded or misfolded proteins in the endoplasmic reticulum (ER) by up-regulating ER-resident enzymes at the transcription level. This is referred to the unfolded protein response (UPR).The UPR takes place in all eukaryotes and is currently the best understood model of inter-organellar signal transduction system. The basic signaling mechanism of the UPR is first illustrated in yeast. A Similar mechanism is now found to be operating in mammalian cells, but it is more complex. Three signaling pathways are involved in the UPR in mammalian cells including Ire1-XBP1, ATF6 and PERK-elF2-phosphated ATF4 pathway. The UPR plays important roles in cellular pathophysiology. Many human diseases including neurodegenerative diseases, diabetes and hyperhomocysteinemia are related to the UPR caused by misfolding of disease proteins. These diseases are also called misfolded protein diseases.
