Pathogenisis Studies of Polyglutamine-Induced Neurodegenerative Diseases |
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| Launch in standalone player | |
| Air date: | Wednesday, February 12, 2003, 3:00:00 PM |
| Category: | Wednesday Afternoon Lectures |
| Description: | The NIH Director's Wednesday Afternoon Lecture Series
Several dominantly inherited spinocerebellar ataxias (SCAs) are caused by expansion of a CAG repeat that encodes glutamine in the respective proteins. Studies in transgenic mice revealed that the mutant protein aggregates in ubiquitin-positive nuclear inclusions (NI) that redistribute molecular chaperones and components of the ubiquitin-proteasome pathway (UPP); further studies in cells, mice, and flies provided convincing evidence that the mutant protein takes on an altered conformation that renders it resistant to degradation and highlighted the importance of the UPP in modifying the phenotype. Impairing ubiquitination aggravates the SCA1 phenotype, for example, while over expressing chaperones mitigates the symptoms. For more information, visit HHMI Lab and Baylor Lab |
| Author: | Huda Y. Zoghbi, M.D., Baylor College of Medicine |
| Runtime: | 65 minutes |
| Rights: | This is a work of the United States Government. No copyright exists on this material. It may be disseminated freely. |
| Download: | Download
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| CIT File ID: | 10181 |
| CIT Live ID: | 1882 |
| Permanent link: | http://videocast.nih.gov/launch.asp?10181 |