Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease

Huntington’s disease (HD) is a rare neurodegenerative disorder caused by an expansion of CAG trinucleotide repeat located in the exon 1 of <i>Huntingtin (HTT)</i> gene in human chromosome 4. The HTT protein is ubiquitously expressed in the brain. Specifically, mutant HTT (mHTT) protein-m...

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Autores principales:	Chaebin Kim, Ali Yousefian-Jazi, Seung-Hye Choi, Inyoung Chang, Junghee Lee, Hoon Ryu
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	Huntington’s disease non-cell autonomous pathway astrocyte oligodendrocyte epigenetics mitochondria dysfunction Biology (General) QH301-705.5 Chemistry QD1-999
Acceso en línea:	https://doaj.org/article/78e121f2f6d54299b5e949e76ae78752
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spelling	oai:doaj.org-article:78e121f2f6d54299b5e949e76ae787522021-11-25T17:57:17ZNon-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease10.3390/ijms2222124991422-00671661-6596https://doaj.org/article/78e121f2f6d54299b5e949e76ae787522021-11-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/22/12499https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067Huntington’s disease (HD) is a rare neurodegenerative disorder caused by an expansion of CAG trinucleotide repeat located in the exon 1 of <i>Huntingtin (HTT)</i> gene in human chromosome 4. The HTT protein is ubiquitously expressed in the brain. Specifically, mutant HTT (mHTT) protein-mediated toxicity leads to a dramatic degeneration of the striatum among many regions of the brain. HD symptoms exhibit a major involuntary movement followed by cognitive and psychiatric dysfunctions. In this review, we address the conventional role of wild type HTT (wtHTT) and how mHTT protein disrupts the function of medium spiny neurons (MSNs). We also discuss how mHTT modulates epigenetic modifications and transcriptional pathways in MSNs. In addition, we define how non-cell autonomous pathways lead to damage and death of MSNs under HD pathological conditions. Lastly, we overview therapeutic approaches for HD. Together, understanding of precise neuropathological mechanisms of HD may improve therapeutic approaches to treat the onset and progression of HD.Chaebin KimAli Yousefian-JaziSeung-Hye ChoiInyoung ChangJunghee LeeHoon RyuMDPI AGarticleHuntington’s diseasenon-cell autonomous pathwayastrocyteoligodendrocyteepigeneticsmitochondria dysfunctionBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 12499, p 12499 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Huntington’s disease non-cell autonomous pathway astrocyte oligodendrocyte epigenetics mitochondria dysfunction Biology (General) QH301-705.5 Chemistry QD1-999
spellingShingle	Huntington’s disease non-cell autonomous pathway astrocyte oligodendrocyte epigenetics mitochondria dysfunction Biology (General) QH301-705.5 Chemistry QD1-999 Chaebin Kim Ali Yousefian-Jazi Seung-Hye Choi Inyoung Chang Junghee Lee Hoon Ryu Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease
description	Huntington’s disease (HD) is a rare neurodegenerative disorder caused by an expansion of CAG trinucleotide repeat located in the exon 1 of <i>Huntingtin (HTT)</i> gene in human chromosome 4. The HTT protein is ubiquitously expressed in the brain. Specifically, mutant HTT (mHTT) protein-mediated toxicity leads to a dramatic degeneration of the striatum among many regions of the brain. HD symptoms exhibit a major involuntary movement followed by cognitive and psychiatric dysfunctions. In this review, we address the conventional role of wild type HTT (wtHTT) and how mHTT protein disrupts the function of medium spiny neurons (MSNs). We also discuss how mHTT modulates epigenetic modifications and transcriptional pathways in MSNs. In addition, we define how non-cell autonomous pathways lead to damage and death of MSNs under HD pathological conditions. Lastly, we overview therapeutic approaches for HD. Together, understanding of precise neuropathological mechanisms of HD may improve therapeutic approaches to treat the onset and progression of HD.
format	article
author	Chaebin Kim Ali Yousefian-Jazi Seung-Hye Choi Inyoung Chang Junghee Lee Hoon Ryu
author_facet	Chaebin Kim Ali Yousefian-Jazi Seung-Hye Choi Inyoung Chang Junghee Lee Hoon Ryu
author_sort	Chaebin Kim
title	Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease
title_short	Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease
title_full	Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease
title_fullStr	Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease
title_full_unstemmed	Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease
title_sort	non-cell autonomous and epigenetic mechanisms of huntington’s disease
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/78e121f2f6d54299b5e949e76ae78752
work_keys_str_mv	AT chaebinkim noncellautonomousandepigeneticmechanismsofhuntingtonsdisease AT aliyousefianjazi noncellautonomousandepigeneticmechanismsofhuntingtonsdisease AT seunghyechoi noncellautonomousandepigeneticmechanismsofhuntingtonsdisease AT inyoungchang noncellautonomousandepigeneticmechanismsofhuntingtonsdisease AT jungheelee noncellautonomousandepigeneticmechanismsofhuntingtonsdisease AT hoonryu noncellautonomousandepigeneticmechanismsofhuntingtonsdisease
_version_	1718411810571288576

Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease

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