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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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
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Abstract 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.
AbstractList 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.
Author Chambers, Joseph E
Marciniak, Stefan J
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  givenname: Joseph E
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  email: jec202@cam.ac.uk, sjm20@cam.ac.uk
  organization: Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Cambridge, United Kingdom jec202@cam.ac.uk sjm20@cam.ac.uk
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  givenname: Stefan J
  surname: Marciniak
  fullname: Marciniak, Stefan J
  organization: Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Cambridge, United Kingdom
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Snippet The endoplasmic reticulum (ER) is a major site of protein synthesis, most strikingly in the specialized secretory cells of metazoans, which can produce their...
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StartPage C657
SubjectTerms Animals
Endoplasmic Reticulum Stress
Endoplasmic Reticulum-Associated Degradation
Heat-Shock Proteins - physiology
Humans
Oxidative Stress
Protein Folding
Proteolysis
Proteostasis Deficiencies - metabolism
Signal Transduction
Title Cellular mechanisms of endoplasmic reticulum stress signaling in health and disease. 2. Protein misfolding and ER stress
URI https://www.ncbi.nlm.nih.gov/pubmed/24944205
Volume 307
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