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 Vol. 307; no. 8; p. C657
Main Authors:	Chambers, Joseph E, Marciniak, Stefan J
Format:	Journal Article
Language:	English
Published:	United States 15-10-2014
Subjects:	Animals Endoplasmic Reticulum Stress Endoplasmic Reticulum-Associated Degradation Heat-Shock Proteins - physiology Humans Oxidative Stress Protein Folding Proteolysis Proteostasis Deficiencies - metabolism Signal Transduction ER stress PERK ATF6 UPR IRE1
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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:	1522-1563
DOI:	10.1152/ajpcell.00183.2014