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Omi / HtrA2 is relevant to the selective vulnerability of striatal neurons in Huntington's disease.

Authors: Inagaki, R  Tagawa, K  Qi, ML  Enokido, Y  Ito, H  Tamura, T  Shimizu, S  Oyanagi, K  Arai, N  Kanazawa, I  Wanker, EE  Okazawa, H 
Citation: Inagaki R, etal., Eur J Neurosci. 2008 Jul;28(1):30-40.
Pubmed: (View Article at PubMed) PMID:18662332
DOI: Full-text: DOI:10.1111/j.1460-9568.2008.06323.x

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.


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CRRD Object Information
CRRD ID: 5688723
Created: 2012-03-02
Species: All species
Last Modified: 2012-03-02
Status: ACTIVE


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