Protein folding stress in neurodegenerative diseases: a glimpse into the ER |
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Authors: | Matus Soledad Glimcher Laurie H Hetz Claudio |
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Affiliation: | 1 Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, Faculty of Medicine, University of Chile, Santiago, Chile;2 Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Chile;3 Department of Immunology and Infectious Diseases, Harvard School of Public Health, USA;4 Department of Medicine, Harvard Medical School, Boston, MA, USA |
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Abstract: | Several neurodegenerative diseases share common neuropathology, primarily featuring the presence in the brain of abnormal protein inclusions containing specific misfolded proteins. Recent evidence indicates that alteration in organelle function is a common pathological feature of protein misfolding disorders, highlighting perturbations in the homeostasis of the endoplasmic reticulum (ER). Signs of ER stress have been detected in most experimental models of neurological disorders and more recently in brain samples from human patients with neurodegenerative disease. To cope with ER stress, cells activate an integrated signaling response termed the unfolded protein response (UPR), which aims to reestablish homeostasis in part through regulation of genes involved in protein folding, quality control and degradation pathways. Here we discuss the particular mechanisms currently proposed to be involved in the generation of protein folding stress in different neurodegenerative conditions and speculate about possible therapeutic interventions. |
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