{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T21:07:22Z","timestamp":1773349642639,"version":"3.50.1"},"reference-count":316,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2021,11,19]],"date-time":"2021-11-19T00:00:00Z","timestamp":1637280000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["NRF-2018M3C7A1056894"],"award-info":[{"award-number":["NRF-2018M3C7A1056894"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["NRF-2020M3E5D9079742"],"award-info":[{"award-number":["NRF-2020M3E5D9079742"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003714","name":"National Research Council of Science and Technology","doi-asserted-by":"publisher","award":["CRC-15-04-KIST"],"award-info":[{"award-number":["CRC-15-04-KIST"]}],"id":[{"id":"10.13039\/501100003714","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003693","name":"Korea Institute of Science and Technology","doi-asserted-by":"publisher","award":["2E30954"],"award-info":[{"award-number":["2E30954"]}],"id":[{"id":"10.13039\/501100003693","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003693","name":"Korea Institute of Science and Technology","doi-asserted-by":"publisher","award":["2E30962"],"award-info":[{"award-number":["2E30962"]}],"id":[{"id":"10.13039\/501100003693","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01AG054156"],"award-info":[{"award-number":["R01AG054156"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01NS109537"],"award-info":[{"award-number":["R01NS109537"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Huntington\u2019s 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.<\/jats:p>","DOI":"10.3390\/ijms222212499","type":"journal-article","created":{"date-parts":[[2021,11,21]],"date-time":"2021-11-21T20:58:21Z","timestamp":1637528301000},"page":"12499","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Non-Cell Autonomous and Epigenetic Mechanisms of Huntington\u2019s Disease"],"prefix":"10.3390","volume":"22","author":[{"given":"Chaebin","family":"Kim","sequence":"first","affiliation":[{"name":"Brain Science Institute, Korea Institute of Science and Technology, Seoul 02792, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali","family":"Yousefian-Jazi","sequence":"additional","affiliation":[{"name":"Brain Science Institute, Korea Institute of Science and Technology, Seoul 02792, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seung-Hye","family":"Choi","sequence":"additional","affiliation":[{"name":"Brain Science Institute, Korea Institute of Science and Technology, Seoul 02792, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Inyoung","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Biology, Boston University, Boston, MA 02215, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junghee","family":"Lee","sequence":"additional","affiliation":[{"name":"Boston University Alzheimer\u2019s Disease Research Center, Boston University, Boston, MA 02118, USA"},{"name":"Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA"},{"name":"VA Boston Healthcare System, Boston, MA 02130, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6544-3732","authenticated-orcid":false,"given":"Hoon","family":"Ryu","sequence":"additional","affiliation":[{"name":"Brain Science Institute, Korea Institute of Science and Technology, Seoul 02792, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,11,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1212\/WNL.94.15_supplement.670","article-title":"Epidemiology of Huntington\u2019s disease (HD) in the US medicare population","volume":"94","author":"Exuzides","year":"2020","journal-title":"Neurology"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"The Huntington\u2019s disease collaborative research group (1993). 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