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http://hdl.handle.net/1942/41571
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DC Field | Value | Language |
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dc.contributor.author | Libberecht, Karen | - |
dc.contributor.author | Vangansewinkel, Tim | - |
dc.contributor.author | Bosch, Ludo Van Den | - |
dc.contributor.author | Lambrichts, Ivo | - |
dc.contributor.author | Wolfs, Esther | - |
dc.date.accessioned | 2023-10-18T13:43:26Z | - |
dc.date.available | 2023-10-18T13:43:26Z | - |
dc.date.issued | 2023 | - |
dc.date.submitted | 2023-10-18T13:41:26Z | - |
dc.identifier.citation | BIOCHEMICAL PHARMACOLOGY, 216 (Art N° 115760) | - |
dc.identifier.issn | 0006-2952 | - |
dc.identifier.uri | http://hdl.handle.net/1942/41571 | - |
dc.description.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. | - |
dc.description.sponsorship | KL is PhD fellow funded by “Fonds Wetenschappelijk Onderzoek” (FWO: “Research Foundation Flanders”; 11A4120N & 11A4122N) and by the “Special Research Fund” (BOF) of Hasselt University (R-10491). TVG is a Junior postdoc fellow funded by “Fonds Wetenschappelijk Onderzoek” (FWO: “Research Foundation Flanders”; 12Z2620N). | - |
dc.language.iso | en | - |
dc.publisher | PERGAMON-ELSEVIER SCIENCE LTD | - |
dc.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/) | - |
dc.subject.other | Charcot-Marie-Tooth disease | - |
dc.subject.other | Protein misfolding | - |
dc.subject.other | Proteostasis | - |
dc.subject.other | Autophagy | - |
dc.subject.other | Schwann cells | - |
dc.subject.other | Neurodegenerative disease | - |
dc.title | Proteostasis plays an important role in demyelinating Charcot Marie Tooth disease | - |
dc.type | Journal Contribution | - |
dc.identifier.volume | 216 | - |
local.format.pages | 11 | - |
local.bibliographicCitation.jcat | A1 | - |
dc.description.notes | Wolfs, E (corresponding author), Hasselt Univ, BIOMED Agoralaan, Bldg C, B-3590 Diepenbeek, Belgium. | - |
dc.description.notes | Karen.libberecht@uhasselt.be; esther.wolfs@uhasselt.be | - |
local.publisher.place | THE BOULEVARD, LANGFORD LANE, KIDLINGTON, OXFORD OX5 1GB, ENGLAND | - |
local.type.refereed | Refereed | - |
local.type.specified | Review | - |
local.bibliographicCitation.artnr | 115760 | - |
dc.identifier.doi | 10.1016/j.bcp.2023.115760 | - |
dc.identifier.pmid | 37604292 | - |
dc.identifier.isi | 001073105600001 | - |
dc.contributor.orcid | Wolfs, Esther/0000-0001-9277-6524 | - |
dc.identifier.eissn | 1873-2968 | - |
local.provider.type | wosris | - |
local.description.affiliation | [Libberecht, Karen; Vangansewinkel, Tim; Wolfs, Esther] UHasselt, Biomed Res Inst BIOMED, Lab Funct Imaging & Res Stem Cells FIERCELab, Diepenbeek, Belgium. | - |
local.description.affiliation | [Bosch, Ludo Van Den] Katholieke Univ Leuven, Dept Neurosci Expt Neurol, Leuven, Belgium. | - |
local.description.affiliation | [Bosch, Ludo Van Den] Leuven Brain Inst LBI, Leuven, Belgium. | - |
local.description.affiliation | [Libberecht, Karen; Vangansewinkel, Tim; Bosch, Ludo Van Den] Ctr Brain & Dis Res, VIB, Lab Neurobiol, Leuven, Belgium. | - |
local.description.affiliation | [Vangansewinkel, Tim; Lambrichts, Ivo] UHasselt, Biomed Res Inst BIOMED, Lab Histol & Regenerat, HISTOREGEN Lab, Diepenbeek, Belgium. | - |
local.description.affiliation | [Wolfs, Esther] Hasselt Univ, BIOMED Agoralaan, Bldg C, B-3590 Diepenbeek, Belgium. | - |
local.uhasselt.international | no | - |
item.accessRights | Open Access | - |
item.fullcitation | Libberecht, Karen; Vangansewinkel, Tim; Bosch, Ludo Van Den; Lambrichts, Ivo & Wolfs, Esther (2023) Proteostasis plays an important role in demyelinating Charcot Marie Tooth disease. In: BIOCHEMICAL PHARMACOLOGY, 216 (Art N° 115760). | - |
item.fulltext | With Fulltext | - |
item.contributor | Libberecht, Karen | - |
item.contributor | Vangansewinkel, Tim | - |
item.contributor | Bosch, Ludo Van Den | - |
item.contributor | Lambrichts, Ivo | - |
item.contributor | Wolfs, Esther | - |
crisitem.journal.issn | 0006-2952 | - |
crisitem.journal.eissn | 1873-2968 | - |
Appears in Collections: | Research publications |
Files in This Item:
File | Description | Size | Format | |
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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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