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Published on: June 7, 2020
ER chaperones in mammalian development and human diseases
1Department of Biochemistry and Molecular Biology, USC/Norris Comprehensive Cancer Center, Keck School of Medicine of the University of Southern California, 1441 Eastlake Ave., Los Angeles, CA 90089-9176, United States.
FEBS Letters
|May 8, 2007
Summary
Mammalian cells adapt to endoplasmic reticulum (ER) stress via molecular chaperones. These proteins, including GRP78/BiP and calnexin, are crucial for ER homeostasis, development, and disease, as shown in new mouse models.
Area of Science:
- Cell Biology
- Molecular Biology
- Biochemistry
Background:
- Endoplasmic reticulum (ER) stress is a cellular response to proteotoxic insults that disrupt ER homeostasis.
- Molecular chaperones within the ER play critical roles in protein folding, quality control, and signaling during ER stress.
- Understanding ER stress response pathways is vital for comprehending cellular adaptation and disease pathogenesis.
Purpose of the Study:
- To review the properties and functions of key ER chaperones and co-chaperones.
- To elucidate the role of these chaperones in cellular adaptation to ER stress.
- To highlight their involvement in mammalian development and various diseases.
Main Methods:
- Literature review synthesizing current knowledge on ER chaperones.
- Analysis of data from genetically modified mouse models with altered chaperone genes.
- Examination of in vivo physiological roles of ER chaperones.
Main Results:
- Detailed summary of properties and functions for GRP78/BiP, GRP94/gp96, GRP170/ORP150, GRP58/ERp57, PDI, ERp72, calnexin, calreticulin, EDEM, Herp, SIL1, and P58(IPK).
- Insights into the physiological relevance of ER chaperones derived from knockout and knock-in mouse models.
- Demonstration of the critical role of ER chaperones in maintaining ER homeostasis and cellular function.
Conclusions:
- ER chaperones are essential regulators of protein homeostasis and signaling during ER stress.
- Dysregulation of ER chaperones contributes to the pathogenesis of various diseases.
- Genetically engineered mouse models provide powerful tools for in vivo investigation of ER chaperone function.
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