https://scholars.lib.ntu.edu.tw/handle/123456789/194985
DC Field | Value | Language |
---|---|---|
dc.contributor | 小兒科 | en |
dc.contributor.author | HWU, WUH-LIANG | en |
dc.contributor.author | HWA, KUO-YUAN | en |
dc.contributor.author | LEE, YU- MAY | en |
dc.creator | 胡務亮;華國媛;李玉梅 | zh_TW |
dc.creator | HWU, WUH-LIANG;HWA, KUO-YUAN;LEE, YU- MAY | en |
dc.date | 2004 | en |
dc.date.accessioned | 2008-12-29T06:19:22Z | - |
dc.date.accessioned | 2018-07-11T17:55:35Z | - |
dc.date.available | 2008-12-29T06:19:22Z | - |
dc.date.available | 2018-07-11T17:55:35Z | - |
dc.date.issued | 2004 | - |
dc.identifier.uri | http://ntur.lib.ntu.edu.tw//handle/246246/94877 | - |
dc.description.abstract | 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. | en |
dc.language | en-us | en |
dc.language.iso | en_US | - |
dc.relation | ANNALS OF NEUROLOGY v.55 n.6 pp.875-878 | en |
dc.relation.ispartof | ANNALS OF NEUROLOGY | - |
dc.subject | GENE | en |
dc.title | Molecular Chaperones Affect Gtp Cyclohydrolase I Mutations in Dopa- Responsive Dystonia | en |
dc.type | journal article | en |
dc.relation.pages | 875-878 | - |
dc.relation.journalvolume | v.55 | - |
dc.relation.journalissue | n.6 | - |
item.languageiso639-1 | en_US | - |
item.cerifentitytype | Publications | - |
item.fulltext | no fulltext | - |
item.openairecristype | http://purl.org/coar/resource_type/c_6501 | - |
item.openairetype | journal article | - |
item.grantfulltext | none | - |
Appears in Collections: | 醫學系 |
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