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Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease
Huntington’s disease (HD) is a neurodegenerative disease characterized by several symptoms encompassing movement, cognition, and behavior. The mutation of the IT15 gene encoding for the huntingtin protein is the cause of HD. Mutant huntingtin interacts with and impairs the function of several transc...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9368941/ https://www.ncbi.nlm.nih.gov/pubmed/35955494 http://dx.doi.org/10.3390/ijms23158363 |
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author | Paldino, Emanuela Fusco, Francesca Romana |
author_facet | Paldino, Emanuela Fusco, Francesca Romana |
author_sort | Paldino, Emanuela |
collection | PubMed |
description | Huntington’s disease (HD) is a neurodegenerative disease characterized by several symptoms encompassing movement, cognition, and behavior. The mutation of the IT15 gene encoding for the huntingtin protein is the cause of HD. Mutant huntingtin interacts with and impairs the function of several transcription factors involved in neuronal survival. Although many mechanisms determining neuronal death have been described over the years, the significant role of inflammation has gained momentum in the last decade. Drugs targeting the elements that orchestrate inflammation have been considered powerful tools to treat HD. In this review, we will describe the data supporting inflammasome and NLRP3 as a target of therapeutics to fight HD, deepening the possible mechanisms of action underlying these effects. |
format | Online Article Text |
id | pubmed-9368941 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-93689412022-08-12 Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease Paldino, Emanuela Fusco, Francesca Romana Int J Mol Sci Review Huntington’s disease (HD) is a neurodegenerative disease characterized by several symptoms encompassing movement, cognition, and behavior. The mutation of the IT15 gene encoding for the huntingtin protein is the cause of HD. Mutant huntingtin interacts with and impairs the function of several transcription factors involved in neuronal survival. Although many mechanisms determining neuronal death have been described over the years, the significant role of inflammation has gained momentum in the last decade. Drugs targeting the elements that orchestrate inflammation have been considered powerful tools to treat HD. In this review, we will describe the data supporting inflammasome and NLRP3 as a target of therapeutics to fight HD, deepening the possible mechanisms of action underlying these effects. MDPI 2022-07-28 /pmc/articles/PMC9368941/ /pubmed/35955494 http://dx.doi.org/10.3390/ijms23158363 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Paldino, Emanuela Fusco, Francesca Romana Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease |
title | Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease |
title_full | Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease |
title_fullStr | Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease |
title_full_unstemmed | Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease |
title_short | Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease |
title_sort | emerging role of nlrp3 inflammasome/pyroptosis in huntington’s disease |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9368941/ https://www.ncbi.nlm.nih.gov/pubmed/35955494 http://dx.doi.org/10.3390/ijms23158363 |
work_keys_str_mv | AT paldinoemanuela emergingroleofnlrp3inflammasomepyroptosisinhuntingtonsdisease AT fuscofrancescaromana emergingroleofnlrp3inflammasomepyroptosisinhuntingtonsdisease |