{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T04:12:13Z","timestamp":1792296733070,"version":"4.4.0"},"reference-count":118,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,12,22]],"date-time":"2022-12-22T00:00:00Z","timestamp":1671667200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Huntington\u2019s disease (HD) is a progressive neurodegenerative disease characterized by preferential loss of neurons in the striatum in patients, which leads to motor and cognitive impairments and death that often occurs 10-15 years after the onset of symptoms. The expansion of a glutamine repeat (&amp;gt;36 glutamines) in the N-terminal region of huntingtin (HTT) has been defined as the cause of HD, but the mechanism underlying neuronal death remains unclear. Multiple mechanisms, including inflammation, may jointly contribute to HD pathogenesis. Altered inflammation response is evident even before the onset of classical symptoms of HD. In this review, we summarize the current evidence on immune and inflammatory changes, from HD animal models to clinical phenomenon of patients with HD. The understanding of the impact of inflammation on HD would help develop novel strategies to treat HD.<\/jats:p>","DOI":"10.3389\/fimmu.2022.1088124","type":"journal-article","created":{"date-parts":[[2022,12,22]],"date-time":"2022-12-22T10:35:02Z","timestamp":1671705302000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":76,"title":["Neuroinflammation in Huntington\u2019s disease: From animal models to clinical therapeutics"],"prefix":"10.3389","volume":"13","author":[{"given":"Qingqing","family":"Jia","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shihua","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiao-Jiang","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peng","family":"Yin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,12,22]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on huntington's disease chromosomes","volume":"72","author":"MacDonald","year":"1993","journal-title":"Cell"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/j.tig.2004.01.008","article-title":"Huntingtin\u2013protein interactions and the pathogenesis of huntington's disease","volume":"20","author":"Li","year":"2004","journal-title":"Trends Genet"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/s1474-4422(09)70178-4","article-title":"Beyond the brain: Widespread pathology in huntington's disease","volume":"8","author":"van der Burg","year":"2009","journal-title":"Lancet Neurol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1146\/annurev.neuro.23.1.217","article-title":"Glutamine repeats and neurodegeneration","volume":"23","author":"Zoghbi","year":"2000","journal-title":"Annu Rev Neurosci"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/541259","article-title":"The role of the immune system in huntington\u2019s disease","volume":"2013","author":"Ellrichmann","year":"2013","journal-title":"Clin Dev Immunol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20080178","article-title":"A novel pathogenic pathway of immune activation detectable before clinical onset in huntington's disease","volume":"205","author":"Bjo\u0308rkqvist","year":"2008","journal-title":"J Exp Med"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/60.2.161","article-title":"Early and progressive accumulation of reactive microglia in the huntington disease brain","volume":"60","author":"Sapp","year":"2001","journal-title":"J Neuropathology Exp Neurol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000222734.56412.17","article-title":"Microglial activation correlates with severity in huntington disease: A clinical and pet study","volume":"66","author":"Pavese","year":"2006","journal-title":"Neurology"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200508072","article-title":"Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity","volume":"171","author":"Shin","year":"2005","journal-title":"J Cell Biol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109.083287","article-title":"Mutant huntingtin in glial cells exacerbates neurological symptoms of huntington disease mice","volume":"285","author":"Bradford","year":"2010","journal-title":"J Biol Chem"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.2174\/18715273113126660170","article-title":"A friend in need may not be a friend indeed: Role of microglia in neurodegenerative diseases","volume":"12","author":"Kaushik","year":"2013","journal-title":"CNS Neurol Disord Drug Targets"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1038\/35099568","article-title":"Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in drosophila","volume":"413","author":"Steffan","year":"2001","journal-title":"Nature"},{"key":"B13","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1007\/978-1-62703-438-8_3","article-title":"Morphometric analysis of huntington\u2019s disease neurodegeneration in drosophila","volume":"1017","author":"Song","year":"2013","journal-title":"Tandem Repeats Genes Proteins Disease. 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