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Review

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

1
Brain Science Institute, Korea Institute of Science and Technology, Seoul 02792, Korea
2
Department of Biology, Boston University, Boston, MA 02215, USA
3
Boston University Alzheimer’s Disease Research Center, Boston University, Boston, MA 02118, USA
4
Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA
5
VA Boston Healthcare System, Boston, MA 02130, USA
*
Authors to whom correspondence should be addressed.
These authors equally contributed to this review.
Academic Editor: Luis M. Valor
Int. J. Mol. Sci. 2021, 22(22), 12499; https://doi.org/10.3390/ijms222212499
Received: 5 October 2021 / Revised: 10 November 2021 / Accepted: 15 November 2021 / Published: 19 November 2021
(This article belongs to the Special Issue Molecular Research on 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 Huntingtin (HTT) 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. View Full-Text
Keywords: Huntington’s disease; non-cell autonomous pathway; astrocyte; oligodendrocyte; epigenetics; mitochondria dysfunction; vesicle trafficking; therapeutic targets Huntington’s disease; non-cell autonomous pathway; astrocyte; oligodendrocyte; epigenetics; mitochondria dysfunction; vesicle trafficking; therapeutic targets
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MDPI and ACS Style

Kim, C.; Yousefian-Jazi, A.; Choi, S.-H.; Chang, I.; Lee, J.; Ryu, H. Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease. Int. J. Mol. Sci. 2021, 22, 12499. https://doi.org/10.3390/ijms222212499

AMA Style

Kim C, Yousefian-Jazi A, Choi S-H, Chang I, Lee J, Ryu H. Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease. International Journal of Molecular Sciences. 2021; 22(22):12499. https://doi.org/10.3390/ijms222212499

Chicago/Turabian Style

Kim, Chaebin, Ali Yousefian-Jazi, Seung-Hye Choi, Inyoung Chang, Junghee Lee, and Hoon Ryu. 2021. "Non-Cell Autonomous and Epigenetic Mechanisms of Huntington’s Disease" International Journal of Molecular Sciences 22, no. 22: 12499. https://doi.org/10.3390/ijms222212499

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