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Insights into White Matter Defect in Huntington’s Disease
Huntington’s disease (HD) is an autosomal-dominant inherited progressive neurodegenerative disorder. It is caused by a CAG repeat expansion in the Huntingtin gene that is translated to an expanded polyglutamine (PolyQ) repeat in huntingtin protein. HD is characterized by mood swings, involuntary mov...
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/PMC9656068/ https://www.ncbi.nlm.nih.gov/pubmed/36359783 http://dx.doi.org/10.3390/cells11213381 |
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author | Sun, Yize Tong, Huichun Yang, Tianqi Liu, Li Li, Xiao-Jiang Li, Shihua |
author_facet | Sun, Yize Tong, Huichun Yang, Tianqi Liu, Li Li, Xiao-Jiang Li, Shihua |
author_sort | Sun, Yize |
collection | PubMed |
description | Huntington’s disease (HD) is an autosomal-dominant inherited progressive neurodegenerative disorder. It is caused by a CAG repeat expansion in the Huntingtin gene that is translated to an expanded polyglutamine (PolyQ) repeat in huntingtin protein. HD is characterized by mood swings, involuntary movement, and cognitive decline in the late disease stage. HD patients often die 15–20 years after disease onset. Currently, there is no cure for HD. Due to the striking neuronal loss in HD, most studies focused on the investigation of the predominantly neuronal degeneration in specific brain regions. However, the pathology of the white matter area in the brains of HD patients was also reported by clinical imaging studies, which showed white matter abnormalities even before the clinical onset of HD. Since oligodendrocytes form myelin sheaths around the axons in the brain, white matter lesions are likely attributed to alterations in myelin and oligodendrocyte-associated changes in HD. In this review, we summarized the evidence for white matter, myelin, and oligodendrocytes alterations that were previously observed in HD patients and animal models. We also discussed potential mechanisms for white matter changes and possible treatment to prevent glial dysfunction in HD. |
format | Online Article Text |
id | pubmed-9656068 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-96560682022-11-15 Insights into White Matter Defect in Huntington’s Disease Sun, Yize Tong, Huichun Yang, Tianqi Liu, Li Li, Xiao-Jiang Li, Shihua Cells Review Huntington’s disease (HD) is an autosomal-dominant inherited progressive neurodegenerative disorder. It is caused by a CAG repeat expansion in the Huntingtin gene that is translated to an expanded polyglutamine (PolyQ) repeat in huntingtin protein. HD is characterized by mood swings, involuntary movement, and cognitive decline in the late disease stage. HD patients often die 15–20 years after disease onset. Currently, there is no cure for HD. Due to the striking neuronal loss in HD, most studies focused on the investigation of the predominantly neuronal degeneration in specific brain regions. However, the pathology of the white matter area in the brains of HD patients was also reported by clinical imaging studies, which showed white matter abnormalities even before the clinical onset of HD. Since oligodendrocytes form myelin sheaths around the axons in the brain, white matter lesions are likely attributed to alterations in myelin and oligodendrocyte-associated changes in HD. In this review, we summarized the evidence for white matter, myelin, and oligodendrocytes alterations that were previously observed in HD patients and animal models. We also discussed potential mechanisms for white matter changes and possible treatment to prevent glial dysfunction in HD. MDPI 2022-10-26 /pmc/articles/PMC9656068/ /pubmed/36359783 http://dx.doi.org/10.3390/cells11213381 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 Sun, Yize Tong, Huichun Yang, Tianqi Liu, Li Li, Xiao-Jiang Li, Shihua Insights into White Matter Defect in Huntington’s Disease |
title | Insights into White Matter Defect in Huntington’s Disease |
title_full | Insights into White Matter Defect in Huntington’s Disease |
title_fullStr | Insights into White Matter Defect in Huntington’s Disease |
title_full_unstemmed | Insights into White Matter Defect in Huntington’s Disease |
title_short | Insights into White Matter Defect in Huntington’s Disease |
title_sort | insights into white matter defect in huntington’s disease |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9656068/ https://www.ncbi.nlm.nih.gov/pubmed/36359783 http://dx.doi.org/10.3390/cells11213381 |
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