RGD Reference Report - Omi / HtrA2 is relevant to the selective vulnerability of striatal neurons in Huntington's disease. - Rat Genome Database

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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.
RGD ID: 5688723
Pubmed: PMID:18662332   (View Abstract at PubMed)
DOI: DOI:10.1111/j.1460-9568.2008.06323.x   (Journal Full-text)

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.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Huntington's disease  ISOHtra2 (Mus musculus)5688723; 5688723protein:increased expression:striatal neuron (mouse)RGD 
Huntington's disease  IEP 5688723protein:increased expression:striatal neuron (mouse)RGD 

Gene Ontology Annotations    Click to see Annotation Detail View

Biological Process
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
neuron apoptotic process  IMP 5688723mutant Htt-induced cell deathRGD 

Objects Annotated

Genes (Rattus norvegicus)
Htra2  (HtrA serine peptidase 2)

Genes (Mus musculus)
Htra2  (HtrA serine peptidase 2)

Genes (Homo sapiens)
HTRA2  (HtrA serine peptidase 2)


Additional Information