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Title: | Proteostasis plays an important role in demyelinating Charcot Marie Tooth disease | Authors: | Libberecht, Karen Vangansewinkel, Tim Bosch, Ludo Van Den Lambrichts, Ivo Wolfs, Esther |
Issue Date: | 2023 | Publisher: | PERGAMON-ELSEVIER SCIENCE LTD | Source: | BIOCHEMICAL PHARMACOLOGY, 216 (Art N° 115760) | Abstract: | Type 1 Charcot-Marie-Tooth disease (CMT1) is the most common demyelinating peripheral neuropathy. Patients suffer from progressive muscle weakness and sensory problems. The underlying disease mechanisms of CMT1 are still unclear and no therapy is currently available, hence patients completely rely on supportive care. Balancing protein levels is a complex multistep process fundamental to maintain cells in their healthy state and a disrupted proteostasis is a hallmark of several neurodegenerative diseases. When protein misfolding occurs, protein quality control systems are activated such as chaperones, the lysosomal-autophagy system and proteasomal degradation to ensure proper degradation. However, in pathological circumstances, these mechanisms are overloaded and thereby become inefficient to clear the load of misfolded proteins. Recent evidence strongly indicates that a disbalance in proteostasis plays an important role in several forms of CMT1. In this review, we present an overview of the protein quality control systems, their role in CMT1, and potential treatment strategies to restore proteostasis. | Notes: | Wolfs, E (corresponding author), Hasselt Univ, BIOMED Agoralaan, Bldg C, B-3590 Diepenbeek, Belgium. Karen.libberecht@uhasselt.be; esther.wolfs@uhasselt.be |
Keywords: | Charcot-Marie-Tooth disease;Protein misfolding;Proteostasis;Autophagy;Schwann cells;Neurodegenerative disease | Document URI: | http://hdl.handle.net/1942/41571 | ISSN: | 0006-2952 | e-ISSN: | 1873-2968 | DOI: | 10.1016/j.bcp.2023.115760 | ISI #: | 001073105600001 | Rights: | 2023 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/bync-nd/4.0/) | Category: | A1 | Type: | Journal Contribution |
Appears in Collections: | Research publications |
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Proteostasis plays an important role in demyelinating Charcot Marie Tooth disease.pdf | Published version | 1.86 MB | Adobe PDF | View/Open |
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