| Title |
Trehalose Attenuates the Gait Ataxia and Gliosis of Spinocerebellar Ataxia Type 17 Mice
|
|---|---|
| Published in |
Neurochemical Research, February 2015
|
| DOI | 10.1007/s11064-015-1530-4 |
| Pubmed ID | |
| Authors |
Zhi-Zhong Chen, Chien-Ming Wang, Guan-Chiun Lee, Ho-Chiang Hsu, Tzu-Ling Wu, Chia-Wei Lin, Chih-Kang Ma, Guey-Jen Lee-Chen, Hei-Jen Huang, Hsiu Mei Hsieh-Li |
| Abstract |
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. |
X Demographics
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United States | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 39 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 39 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Bachelor | 6 | 15% |
| Student > Ph. D. Student | 4 | 10% |
| Student > Doctoral Student | 3 | 8% |
| Other | 3 | 8% |
| Researcher | 3 | 8% |
| Other | 5 | 13% |
| Unknown | 15 | 38% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 5 | 13% |
| Biochemistry, Genetics and Molecular Biology | 3 | 8% |
| Neuroscience | 3 | 8% |
| Chemistry | 3 | 8% |
| Agricultural and Biological Sciences | 2 | 5% |
| Other | 8 | 21% |
| Unknown | 15 | 38% |
Attention Score in Context
This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 13 February 2015.
All research outputs
#20,259,845
of 22,789,566 outputs
Outputs from Neurochemical Research
#1,689
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Outputs of similar age
#301,284
of 357,823 outputs
Outputs of similar age from Neurochemical Research
#13
of 22 outputs
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