The ER (endoplasmic reticulum) is the protein folding ‘factory’ of the secretory pathway. Virtually all proteins destined for the plasma membrane, the extracellular space or other secretory compartments undergo folding and maturation within the ER. The ER hosts a unique PQC (protein quality control) system that allows specialized modifications such as glycosylation and disulfide bond formation essential for the correct folding and function of many secretory proteins. It is also the major checkpoint for misfolded or aggregation-prone proteins that may be toxic to the cell or extracellular environment. A failure of this system, due to aging or other factors, has therefore been implicated in a number of serious human diseases. In this article, we discuss several key features of ER PQC that maintain the health of the cellular secretome.
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October 2016
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Patricija van Oosten-Hawle
Patricija van Oosten-Hawle
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A schematic depiction of protease network in mitochondria. In this issue, Voos et al. review mitochondrial protein quality control, looking at the biochemical processes and the enzymatic components that are responsible for maintaining mitochondrial protein homeostasis; see pages 213–225. - PDF Icon PDF LinkTable of Contents
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Review Article|
October 15 2016
Protein quality control at the endoplasmic reticulum
Kathleen McCaffrey;
Kathleen McCaffrey
*
1Cellular Protein Chemistry, Utrecht University, 3584CH Padualaan 8, 3584 CH Utrecht, The Netherlands
*Correspondence: Kathleen McCaffrey (email k.mccaffrey@uu.nl).
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Ineke Braakman
Ineke Braakman
1Cellular Protein Chemistry, Utrecht University, 3584CH Padualaan 8, 3584 CH Utrecht, The Netherlands
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Publisher: Portland Press Ltd
Received:
January 14 2016
Revision Received:
April 30 2016
Accepted:
May 31 2016
Online ISSN: 1744-1358
Print ISSN: 0071-1365
© 2016 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society
2016
Essays Biochem (2016) 60 (2): 227–235.
Article history
Received:
January 14 2016
Revision Received:
April 30 2016
Accepted:
May 31 2016
Citation
Patricija van Oosten-Hawle, Kathleen McCaffrey, Ineke Braakman; Protein quality control at the endoplasmic reticulum. Essays Biochem 15 October 2016; 60 (2): 227–235. doi: https://doi.org/10.1042/EBC20160003
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