Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Sun, Yize, Tong, Huichun, Yang, Tianqi, Liu, Li, Li, Xiao-Jiang, Li, Shihua
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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
_version_ 1784829341775953920
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
work_keys_str_mv AT sunyize insightsintowhitematterdefectinhuntingtonsdisease
AT tonghuichun insightsintowhitematterdefectinhuntingtonsdisease
AT yangtianqi insightsintowhitematterdefectinhuntingtonsdisease
AT liuli insightsintowhitematterdefectinhuntingtonsdisease
AT lixiaojiang insightsintowhitematterdefectinhuntingtonsdisease
AT lishihua insightsintowhitematterdefectinhuntingtonsdisease