SUMO-modifying Huntington's disease.
Autor: | Soares ES; Post-graduate Program in Pharmacology, Federal University of Santa Catarina (UFSC), Florianópolis, Santa Catarina, Brazil., Prediger RD; Post-graduate Program in Pharmacology, Federal University of Santa Catarina (UFSC), Florianópolis, Santa Catarina, Brazil.; Post-graduate Program in Neuroscience, UFSC, Florianópolis, Santa Catarina, Brazil., Brocardo PS; Post-graduate Program in Neuroscience, UFSC, Florianópolis, Santa Catarina, Brazil., Cimarosti HI; Post-graduate Program in Pharmacology, Federal University of Santa Catarina (UFSC), Florianópolis, Santa Catarina, Brazil.; Post-graduate Program in Neuroscience, UFSC, Florianópolis, Santa Catarina, Brazil. |
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Jazyk: | angličtina |
Zdroj: | IBRO neuroscience reports [IBRO Neurosci Rep] 2022 Mar 09; Vol. 12, pp. 203-209. Date of Electronic Publication: 2022 Mar 09 (Print Publication: 2022). |
DOI: | 10.1016/j.ibneur.2022.03.002 |
Abstrakt: | Small ubiquitin-like modifiers, SUMOs, are proteins that are conjugated to target substrates and regulate their functions in a post-translational modification called SUMOylation. In addition to its physiological roles, SUMOylation has been implicated in several neurodegenerative diseases, such as Alzheimer's, Parkinson's, and Huntington's diseases (HD). HD is a neurodegenerative monogenetic autosomal dominant disorder caused by a mutation in the CAG repeat of the huntingtin ( htt ) gene, which expresses a mutant Htt protein more susceptible to aggregation and toxicity. Besides Htt, other SUMO ligases, enzymes, mitochondrial and autophagic components are also important for the progression of the disease. Here we review the main aspects of Htt SUMOylation and its role in cellular processes involved in the pathogenesis of HD. (© 2022 The Authors.) |
Databáze: | MEDLINE |
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