title: Cellular mechanisms of endoplasmic reticulum stress signaling in health and disease. 2. Protein misfolding and ER stress.
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.
authors.affiliation: Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Cambridge, United Kingdom jec202@cam.ac.uk sjm20@cam.ac.uk.
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Chambers
authors.affiliation: Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Cambridge, United Kingdom.
authors.forename: Stefan J
authors.initials: SJ
authors.lastname: Marciniak
mesh_terms: D000818:Animals; D059865:Endoplasmic Reticulum Stress; D060746:Endoplasmic Reticulum-Associated Degradation; D006360:Heat-Shock Proteins / Q000502:physiology; D006801:Humans; D018384:Oxidative Stress; D017510:Protein Folding; D059748:Proteolysis; D057165:Proteostasis Deficiencies / Q000378:metabolism; D015398:Signal Transduction
chemical_list: D006360:Heat-Shock Proteins
keywords: ATF6; ER stress; IRE1; PERK; UPR