Ubiquitin system mutations in neurological diseases Review
| Authors: | Zenge, C.; Ordureau, A. |
| Review Title: | Ubiquitin system mutations in neurological diseases |
| Abstract: | Neuronal ubiquitin balance impacts the fate of countless cellular proteins, and its disruption is associated with various neurological disorders. The ubiquitin system is critical for proper neuronal cell state transitions and the clearance of misfolded or aggregated proteins that threaten cellular integrity. This article reviews the state of and recent advancements in our understanding of the disruptions to components of the ubiquitin system, in particular E3 ligases and deubiquitylases, in neurodevelopmental and neurodegenerative diseases. Specific focus is on enzymes with recent progress in their characterization, including identifying enzyme-substrate pairs, the use of stem cell and animal models, and the development of therapeutics for ubiquitin-related diseases. © 2024 Elsevier Ltd |
| Keywords: | mutation; review; nonhuman; ubiquitin; animal model; enzyme substrate; ubiquitin protein ligase e3; neurologic disease; nerve cell differentiation; nerve cell; degenerative disease; neuropathology; nerve degeneration; neurodegeneration; neurodevelopment; e3 ubiquitin ligases; motor neuron disease; human; deubiquitylase (dub); neuropathologies |
| Journal Title: | Trends in Biochemical Sciences |
| Volume: | 49 |
| Issue: | 10 |
| ISSN: | 0968-0004 |
| Publisher: | Elsevier Inc. |
| Date Published: | 2024-10-01 |
| Start Page: | 875 |
| End Page: | 887 |
| Language: | English |
| DOI: | 10.1016/j.tibs.2024.06.011 |
| PUBMED: | 38972780 |
| PROVIDER: | scopus |
| PMCID: | PMC11455613 |
| DOI/URL: | |
| Notes: | Review -- MSK corresponding author is Alban Ordureau -- Source: Scopus |
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