https://scholars.lib.ntu.edu.tw/handle/123456789/194985
標題: | Molecular Chaperones Affect Gtp Cyclohydrolase I Mutations in Dopa- Responsive Dystonia | 作者: | HWU, WUH-LIANG HWA, KUO-YUAN LEE, YU- MAY |
關鍵字: | GENE | 公開日期: | 2004 | 卷: | v.55 | 期: | n.6 | 起(迄)頁: | 875-878 | 來源出版物: | ANNALS OF NEUROLOGY | 摘要: | Unstable GTP cyclohydrolase I (GCH) mutations in dopa- responsive dystonia (DRD) can exert a dominant-negative effect in the HeLa cell model , but in a batch of cells this effect could not be shown. Through differential display, we found a higher Hsc70 expression in the non- dominant-negative cells. We further demonstrated that ectopic expression of Hsp40/Hsp70 stabilized the GCH mutant G201E. Moreover, Hsp90 inhibitor geldanamycin destroyed the wild-type GCH level, and heat shock increased the synthesis of GCH protein. Therefore, the dominant-negative effect produced by unstable proteins would be susceptible to the status of molecular chaperones, which could be the modifying genes and therapeutic targets for DRD and other genetic diseases. |
URI: | http://ntur.lib.ntu.edu.tw//handle/246246/94877 |
顯示於: | 醫學系 |
在 IR 系統中的文件,除了特別指名其著作權條款之外,均受到著作權保護,並且保留所有的權利。