Cellular mechanisms of endoplasmic reticulum stress signaling in health and disease. 2. Protein misfolding and ER stress

The endoplasmic reticulum (ER) is a major site of protein synthesis, most strikingly in the specialized secretory cells of metazoans, which can produce their own weight in proteins daily. Cells possess a diverse machinery to ensure correct folding, assembly, and secretion of proteins from the ER. Wh...

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Bibliographic Details
Published in:American Journal of Physiology: Cell Physiology 2014-10, Vol.307 (8), p.C657-C670
Main Authors: Chambers, Joseph E, Marciniak, Stefan J
Format: Article
Language:English
Subjects:
Animals
Cells
Disease
Endoplasmic Reticulum Stress
Endoplasmic Reticulum-Associated Degradation
Health
Heat-Shock Proteins - physiology
Humans
Oxidative Stress
Protein Folding
Protein synthesis
Proteolysis
Proteostasis Deficiencies - metabolism
Signal Transduction
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Summary:The endoplasmic reticulum (ER) is a major site of protein synthesis, most strikingly in the specialized secretory cells of metazoans, which can produce their own weight in proteins daily. Cells possess a diverse machinery to ensure correct folding, assembly, and secretion of proteins from the ER. When this machinery is overwhelmed, the cell is said to experience ER stress, a result of the accumulation of unfolded or misfolded proteins in the lumen of the organelle. Here we discuss the causes of ER stress and the mechanisms by which cells elicit a response, with an emphasis on recent discoveries.
ISSN:0363-6143
1522-1563
DOI:10.1152/ajpcell.00183.2014