التفاصيل البيبلوغرافية
| العنوان: |
Chorein deficiency leads to upregulation of gephyrin and GABAA receptor |
| المؤلفون: |
Kurano, Yutaka1, Nakamura, Masayuki1, Ichiba, Mio1, Matsuda, Mieko1, Mizuno, Emiko1, Kato, Maiko1, Izumo, Shuji2, Sano, Akira1 sano@m3.kufm.kagoshima-u.ac.jp |
| المصدر: |
Biochemical & Biophysical Research Communications. Dec2006, Vol. 351 Issue 2, p438-442. 5p. |
| مصطلحات موضوعية: |
*GABA, *GENE expression, *NEURODEGENERATION, *GENETICS |
| مستخلص: |
Abstract: Chorea-acanthocytosis (ChAc) is a hereditary neurodegenerative disorder caused by loss of function mutations in the VPS13A gene encoding chorein. Recently, using a gene-targeting technique to delete exons 60–61, we produced a ChAc-model mouse that corresponds to a human disease mutation. In this study, a comparative microarray analysis of gene expression in the striatum revealed an increased level of gephyrin gene expression in the ChAc-model mice compared with wild type mice. Since gephyrin is known as a GABAA receptor-anchoring protein, we compared the protein-level expression and localization of gephyrin and the GABAA receptor α1 (GABRA1) and γ2 (GABRG2) subunits. Gephyrin and GABRG2 immunoreactivities in the striatum and hippocampus of the ChAc-model mice were significantly higher than those in the wild types. Our results suggest that chorein functional loss may lead to a compensatory upregulation of gephyrin and GABRG2 in the pathologic condition in ChAc. [Copyright &y& Elsevier] |
| قاعدة البيانات: |
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