2012 Fiscal Year Final Research Report
Elucidation of the mechanisms underlying in the immunological abnormalities of lysosomal storage diseases
| Project/Area Number |
23790448
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| Research Category |
Grant-in-Aid for Young Scientists (B)
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| Allocation Type | Multi-year Fund |
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| Research Field |
Experimental pathology
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| Research Institution | Yokohama City University |
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Principal Investigator |
YAMAGUCHI Akira 横浜市立大学, 横浜市立大学, 客員研究員 (20381585)
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| Project Period (FY) |
2011 – 2012
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| Keywords | ライソゾーム病 / 自己抗体 / 炎症反応 / ケモカイン |
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| Research Abstract |
We have previously found that the progress neurologic disease induced in SD and hex-/- mice, is associated with the production of pathogenic anti-glycolipid autoantibodies. In this study, we focused immunological abnormalities in the CNS. We employed Real-time PCR analysis to monitor gene expression in the terminal stage of hexb-/- mice and found that gene associated with the immune responses were up-regulated. B lymphocyte chemoattractant CXCL-13 was one of there up-regulated genes and is expressed specifically in the CNS. To determine the role of the CXCL-13, the cxcl-13 gene was additionally disrupted in hexb-/- mice, as it play a key role in the autoimmune disease. Clinical symptoms were improved and life spans were extended in the hexb-/-, cxcl-13-/- mice and the number of neuronal cells was also increased than hexb-/- mice. These findings suggest that the expression of CXCL-13 plays an important role in the pathogenesis of neuropathy in hexb-/- mice and therefore provides a target for novel therapies.
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