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Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies

Huntington's disease (HD) is a neurodegenerative disorder characterized by selective loss of neurons in the striatum and cortex, which leads to progressive motor dysfunction, cognitive decline, and psychiatric disorders. Although the cause of HD is well described—HD is a genetic disorder caused...

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Autores principales: Rocha, Natalia P., Ribeiro, Fabiola M., Furr-Stimming, Erin, Teixeira, Antonio L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4992798/
https://www.ncbi.nlm.nih.gov/pubmed/27578922
http://dx.doi.org/10.1155/2016/8653132
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author Rocha, Natalia P.
Ribeiro, Fabiola M.
Furr-Stimming, Erin
Teixeira, Antonio L.
author_facet Rocha, Natalia P.
Ribeiro, Fabiola M.
Furr-Stimming, Erin
Teixeira, Antonio L.
author_sort Rocha, Natalia P.
collection PubMed
description Huntington's disease (HD) is a neurodegenerative disorder characterized by selective loss of neurons in the striatum and cortex, which leads to progressive motor dysfunction, cognitive decline, and psychiatric disorders. Although the cause of HD is well described—HD is a genetic disorder caused by a trinucleotide (CAG) repeat expansion in the gene encoding for huntingtin (HTT) on chromosome 4p16.3—the ultimate cause of neuronal death is still uncertain. Apart from impairment in systems for handling abnormal proteins, other metabolic pathways and mechanisms might contribute to neurodegeneration and progression of HD. Among these, inflammation seems to play a role in HD pathogenesis. The current review summarizes the available evidence about immune and/or inflammatory changes in HD. HD is associated with increased inflammatory mediators in both the central nervous system and periphery. Accordingly, there have been some attempts to slow HD progression targeting the immune system.
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spelling pubmed-49927982016-08-30 Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies Rocha, Natalia P. Ribeiro, Fabiola M. Furr-Stimming, Erin Teixeira, Antonio L. Mediators Inflamm Review Article Huntington's disease (HD) is a neurodegenerative disorder characterized by selective loss of neurons in the striatum and cortex, which leads to progressive motor dysfunction, cognitive decline, and psychiatric disorders. Although the cause of HD is well described—HD is a genetic disorder caused by a trinucleotide (CAG) repeat expansion in the gene encoding for huntingtin (HTT) on chromosome 4p16.3—the ultimate cause of neuronal death is still uncertain. Apart from impairment in systems for handling abnormal proteins, other metabolic pathways and mechanisms might contribute to neurodegeneration and progression of HD. Among these, inflammation seems to play a role in HD pathogenesis. The current review summarizes the available evidence about immune and/or inflammatory changes in HD. HD is associated with increased inflammatory mediators in both the central nervous system and periphery. Accordingly, there have been some attempts to slow HD progression targeting the immune system. Hindawi Publishing Corporation 2016 2016-08-08 /pmc/articles/PMC4992798/ /pubmed/27578922 http://dx.doi.org/10.1155/2016/8653132 Text en Copyright © 2016 Natalia P. Rocha et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Rocha, Natalia P.
Ribeiro, Fabiola M.
Furr-Stimming, Erin
Teixeira, Antonio L.
Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies
title Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies
title_full Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies
title_fullStr Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies
title_full_unstemmed Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies
title_short Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies
title_sort neuroimmunology of huntington's disease: revisiting evidence from human studies
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4992798/
https://www.ncbi.nlm.nih.gov/pubmed/27578922
http://dx.doi.org/10.1155/2016/8653132
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