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| Item Type: | Article |
|---|---|
| Title: | Omi / HtrA2 is relevant to the selective vulnerability of striatal neurons in Huntington's disease |
| Creators Name: | Inagaki, R., Tagawa, K., Qi, M.L., Enokido, Y., Ito, H., Tamura, T., Shimizu, S., Oyanagi, K., Arai, N., Kanazawa, I., Wanker, E.E. and Okazawa, H. |
| Abstract: | Selective vulnerability of neurons is a critical feature of neurodegenerative diseases, but the underlying molecular mechanisms remain largely unknown. We here report that Omi/HtrA2, a mitochondrial protein regulating survival and apoptosis of cells, decreases selectively in striatal neurons that are most vulnerable to the Huntington's disease (HD) pathology. In microarray analysis, Omi/HtrA2 was decreased under the expression of mutant huntingtin (htt) in striatal neurons but not in cortical or cerebellar neurons. Mutant ataxin-1 (Atx-1) did not affect Omi/HtrA2 in any type of neuron. Western blot analysis of primary neurons expressing mutant htt also confirmed the selective reduction of the Omi/HtrA2 protein. Immunohistochemistry with a mutant htt-transgenic mouse line and human HD brains confirmed reduction of Omi/HtrA2 in striatal neurons. Overexpression of Omi/HtrA2 by adenovirus vector reverted mutant htt-induced cell death in primary neurons. These results collectively suggest that the homeostatic but not proapoptotic function of Omi/HtrA2 is linked to selective vulnerability of striatal neurons in HD pathology. |
| Keywords: | Cell Death, Huntingtin, Huntington's Disease, Mitochondria, Omi/HtrA2, Polyglutamine, Animals, Mice, Rats |
| Source: | European Journal of Neuroscience |
| ISSN: | 0953-816X |
| Publisher: | Blackwell Publishing |
| Volume: | 28 |
| Number: | 1 |
| Page Range: | 30-40 |
| Date: | July 2008 |
| Official Publication: | https://doi.org/10.1111/j.1460-9568.2008.06323.x |
| PubMed: | View item in PubMed |
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