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role of BimEL in the pathogenesis of Huntington's disease
| Title: | The role of BimEL in the pathogenesis of Huntington's disease. |
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| Name(s): |
Leon, Rebecca Charles E. Schmidt College of Science Department of Biological Sciences |
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| Type of Resource: | text | |
| Genre: | Electronic Thesis Or Dissertation | |
| Date Issued: | 2012 | |
| Publisher: | Florida Atlantic University | |
| Physical Form: | electronic | |
| Extent: | ix, 101 p. : ill. (some col.) | |
| Language(s): | English | |
| Summary: | Huntington's Disease (HD) is a devastating neurodegenerative disorder caused by an expanded polyglutamine repeat within the Huntingtin gene IT15. In this study we demonstrated that Bcl-2 interacting mediator of cell death Extra Long (BimEL) protein expression was significantly increased in cells expressing mutant Huntingtin (mHtt). Moreover, striatal BimEL expression remained high in an R6/2 HD mouse model throughout the disease progression. Utilizing novel BimEL phospho-mutants we demonstrated the phosphorylation of Ser65 to be important for the stabilization of BimEL. We provided evidence that impaired proteasome function, increased JNK activity and reduced striatal BDNF lead to changes in the phosphorylation of BimEL, thereby promoting its stabilization specifically within the striatum of R6/2 mice. Furthermore, knocking down BimEL expression prevented mHtt-induced cell death in a HD cell culture. Taken together, these findings suggest that BimEL may contribute to the selective neurodegeneration and pathogenesis of HD. | |
| Identifier: | 820353509 (oclc), 3355556 (digitool), FADT3355556 (IID), fau:3927 (fedora) | |
| Note(s): |
by Rebecca Leon. Thesis (Ph.D.)--Florida Atlantic University, 2012. Includes bibliography. Mode of access: World Wide Web. System requirements: Adobe Reader. |
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| Subject(s): |
Huntington's chorea -- Pathophysiology Huntington's chorea -- Molecular aspects Huntington's chorea -- Genetic aspects Nervous system -- Degeneration -- Pathophysiology Nervous system -- Degeneration -- Molecular aspects Glutamine -- Pathophysiology |
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| Persistent Link to This Record: | http://purl.flvc.org/FAU/3355556 | |
| Use and Reproduction: | http://rightsstatements.org/vocab/InC/1.0/ | |
| Host Institution: | FAU |
