Ubiquitin-binding site 1 of pathogenic ataxin-3 regulates its toxicity in
CAG triplet repeat
Drosophila
ataxia
misfolding and aggregation
neurodegeneration
polyglutamine (polyQ)
ubiquitin
Journal
Frontiers in neuroscience
ISSN: 1662-4548
Titre abrégé: Front Neurosci
Pays: Switzerland
ID NLM: 101478481
Informations de publication
Date de publication:
2022
2022
Historique:
received:
30
11
2022
accepted:
21
12
2022
entrez:
3
2
2023
pubmed:
4
2
2023
medline:
4
2
2023
Statut:
epublish
Résumé
Spinocerebellar Ataxia Type 3 (SCA3) is a member of the family of polyglutamine (polyQ) diseases that are caused by anomalous CAG triplet repeat expansions in several genes. SCA3 results from abnormal polyQ expansion in the deubiquitinase (DUB), ataxin-3 (Atxn3). To understand the role of the different domains of mutant Atxn3 on its pathogenicity, with the hope that they can be explored for therapeutic interventions, we have systematically studied their individual and collective effects on its toxicity. One such domain is ubiquitin-binding site 1 (UbS1) on the catalytic domain of Atxn3; UbS1 is necessary for the enzymatic activity of Atxn3. Here, we investigated the importance of UbS1 on the toxicity of pathogenic Atxn3. We generated transgenic
Identifiants
pubmed: 36733922
doi: 10.3389/fnins.2022.1112688
pmc: PMC9887036
doi:
Types de publication
Journal Article
Langues
eng
Pagination
1112688Informations de copyright
Copyright © 2023 Prifti, Libohova, Harris, Tsou and Todi.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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