The UPR in Neurodegenerative Disease: Not Just an Inside Job
1
Department of Clinical Genetics, Amsterdam University Medical Centers location VUmc, 1081 HV Amsterdam, The Netherlands
2
Department of Functional Genomics, Center for Neurogenomics and Cognitive Research, Vrije Universiteit (VU), 1081 HV Amsterdam, The Netherlands
*
Author to whom correspondence should be addressed.
Biomolecules 2020, 10(8), 1090; https://doi.org/10.3390/biom10081090
Received: 26 May 2020
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Revised: 16 July 2020
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Accepted: 20 July 2020
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Published: 22 July 2020
(This article belongs to the Special Issue Endoplasmic Reticulum Stress in Diseases)
Neurons are highly specialized cells that continuously and extensively communicate with other neurons, as well as glia cells. During their long lifetime, the post-mitotic neurons encounter many stressful situations that can disrupt protein homeostasis (proteostasis). The importance of tight protein quality control is illustrated by neurodegenerative disorders where disturbed neuronal proteostasis causes neuronal dysfunction and loss. For their unique function, neurons require regulated and long-distance transport of membrane-bound cargo and organelles. This highlights the importance of protein quality control in the neuronal endomembrane system, to which the unfolded protein response (UPR) is instrumental. The UPR is a highly conserved stress response that is present in all eukaryotes. However, recent studies demonstrate the existence of cell-type-specific aspects of the UPR, as well as cell non-autonomous UPR signaling. Here we discuss these novel insights in view of the complex cellular architecture of the brain and the implications for neurodegenerative diseases.
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Keywords:
unfolded protein response; unconventional secretion; neurodegenerative disease; proteostasis; cell non-autonomous
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MDPI and ACS Style
van Ziel, A.M.; Scheper, W. The UPR in Neurodegenerative Disease: Not Just an Inside Job. Biomolecules 2020, 10, 1090. https://doi.org/10.3390/biom10081090
AMA Style
van Ziel AM, Scheper W. The UPR in Neurodegenerative Disease: Not Just an Inside Job. Biomolecules. 2020; 10(8):1090. https://doi.org/10.3390/biom10081090
Chicago/Turabian Stylevan Ziel, Anna M., and Wiep Scheper. 2020. "The UPR in Neurodegenerative Disease: Not Just an Inside Job" Biomolecules 10, no. 8: 1090. https://doi.org/10.3390/biom10081090
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