iNKT Cell Activation Exacerbates the Development of Huntington's Disease in R6/2 Transgenic Mice
| العنوان: | iNKT Cell Activation Exacerbates the Development of Huntington's Disease in R6/2 Transgenic Mice |
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| المؤلفون: | Sung Won Lee, Luc Van Kaer, Seokmann Hong, Manho Kim, Hyun Jung Park, Wooseok Im |
| المصدر: | Mediators of Inflammation Mediators of Inflammation, Vol 2019 (2019) |
| بيانات النشر: | Hindawi, 2019. |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | Genetically modified mouse, Huntingtin, Article Subject, Genotype, Immunology, Stimulation, Galactosylceramides, Biology, Lymphocyte Activation, Pathogenesis, Mice, Immune system, Huntington's disease, medicine, lcsh:Pathology, Leukocytes, Animals, Interleukin 4, Mice, Knockout, Brain, Cell Biology, medicine.disease, Disease Models, Animal, Huntington Disease, Disease Progression, Cytokines, Natural Killer T-Cells, Cell activation, lcsh:RB1-214, Research Article |
| الوصف: | Huntington’s disease (HD) is an inherited neurodegenerative disorder which is caused by a mutation of the huntingtin (HTT) gene. Although the pathogenesis of HD has been associated with inflammatory responses, if and how the immune system contributes to the onset of HD is largely unknown. Invariant natural killer T (iNKT) cells are a group of innate-like regulatory T lymphocytes that can rapidly produce various cytokines such as IFNγ and IL4 upon stimulation with the glycolipid α-galactosylceramide (α-GalCer). By employing both R6/2 Tg mice (murine HD model) and Jα18 KO mice (deficient in iNKT cells), we investigated whether alterations of iNKT cells affect the development of HD in R6/2 Tg mice. We found that Jα18 KO R6/2 Tg mice showed disease progression comparable to R6/2 Tg mice, indicating that the absence of iNKT cells did not have any significant effects on HD development. However, repeated activation of iNKT cells with α-GalCer facilitated HD progression in R6/2 Tg mice, and this was associated with increased infiltration of iNKT cells in the brain. Taken together, our results demonstrate that repeated α-GalCer treatment of R6/2 Tg mice accelerates HD progression, suggesting that immune activation can affect the severity of HD pathogenesis. |
| وصف الملف: | text/xhtml |
| اللغة: | English |
| تدمد: | 1466-1861 0962-9351 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::716fe2824a328ad0247633ee3f7afd8fTest http://europepmc.org/articles/PMC6350536Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....716fe2824a328ad0247633ee3f7afd8f |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14661861 09629351 |
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