Improvement of peripheral neuropathy by Adeno-associated virus vector expressing a-galactosidase A in murine Fabry model
| Project/Area Number |
15K19637
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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 |
Pediatrics
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| Research Institution | Jikei University School of Medicine |
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Principal Investigator |
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| Research Collaborator |
OHASHI Toya
KOBAYASHI Hiroshi
SHIMADA Yohta
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| Project Period (FY) |
2015-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2017: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2016: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | アンダーソン・ファブリー病 / 末梢神経症状 / 痛み / ファブリ病 / 疼痛 / Gb3 / Anderson-Fabry Disease / α-galactosidase A / globotriaosylceramide / TRP channel / dorsal root ganglion / ファブリー病 / 末梢神経障害 |
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| Outline of Final Research Achievements |
Anderson-Fabry disease (FD) is a lysosomal storage disorder caused by mutation of the alpha-galactosidase A (GLA) gene. Many FD patients have peripheral neuropathy in the childhoods. This is caused by accumulation of globotoriaosylceramide (Gb3) in dorsal root ganglia (DRG). FD murine model expressed Trpv1 mRNA. The express level was same level as wild-type (WT) mice. Unexpectedly, FD mouse did not show pain symptoms in this experiment. FD mouse showed hyposensitivity to the hot-plate test compare to WT mouse. There were many myelin-like granules in the cytosol of FD DRG neuron. However there was not detected in the axon. It was difficult to observe “patient-like” peripheral neuropathy in this murine model. Our data could not clear the relationship between TRPs and peripheral nerve symptoms.
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Report
(4 results)
Research Products
(4 results)
