Trehalose Attenuates the Gait Ataxia and Gliosis of Spinocerebellar Ataxia Type 17 Mice
| العنوان: | Trehalose Attenuates the Gait Ataxia and Gliosis of Spinocerebellar Ataxia Type 17 Mice |
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| المؤلفون: | Tzu Ling Wu, Zhi Zhong Chen, Guey Jen Lee-Chen, Guan Chiun Lee, Chih Kang Ma, Ho Chiang Hsu, Hei Jen Huang, Chien Ming Wang, Chia Wei Lin, Hsiu Mei Hsieh-Li |
| المصدر: | Neurochemical Research. 40:800-810 |
| بيانات النشر: | Springer Science and Business Media LLC, 2015. |
| سنة النشر: | 2015 |
| مصطلحات موضوعية: | Blood Glucose, Gait Ataxia, Male, Genetically modified mouse, Mice, Transgenic, Biology, Protein aggregation, Biochemistry, Mice, Purkinje Cells, Cellular and Molecular Neuroscience, chemistry.chemical_compound, medicine, Animals, Spinocerebellar Ataxias, Gliosis, Body Weight, Trehalose, General Medicine, medicine.disease, Cell biology, Cytosol, chemistry, Spinocerebellar ataxia, medicine.symptom, Trinucleotide repeat expansion |
| الوصف: | Spinocerebellar ataxia type 17 (SCA17) is caused by CAG/CAA repeat expansion on the gene encoding a general transcription factor, TATA-box-binding protein (TBP). The CAG repeat expansion leads to the reduced solubility of polyglutamine TBP and induces aggregate formation. The TBP aggregation, mostly present in the cell nuclei, is distinct from that in most other neurodegenerative diseases, in which the aggregation is formed in cytosol or extracellular compartments. Trehalose is a disaccharide issued by the Food and Drug Administration with a Generally Recognized As Safe status. Lines of evidence suggest trehalose could prevent protein aggregate formation in several neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and Huntington's disease. In this study, we evaluated the therapeutic potential of trehalose on SCA17 using cerebellar primary and organotypic culture systems and a mouse model. Our results showed that TBP nuclear aggregation was significantly decreased in both the primary and slice cultures. Trehalose (4 %) was further supplied in the drinking water of SCA17 transgenic mice. We found both the gait behavior in the footprint analysis and motor coordination in the rotarod task were significantly improved in the trehalose-treated SCA17 mice. The cerebellar weight was increased and the astrocyte gliosis was reduced in SCA17 mice after trehalose treatment. These data suggest that trehalose could be a potential nontoxic treatment for SCA17. |
| تدمد: | 1573-6903 0364-3190 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::532d732035c005fb4a2c8e03e9fc9939Test https://doi.org/10.1007/s11064-015-1530-4Test |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....532d732035c005fb4a2c8e03e9fc9939 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15736903 03643190 |
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