Study of theretinal ganglion cell specific gene, optic atrophy, and gene threrapy
| Project/Area Number |
12470368
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Ophthalmology
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| Research Institution | Keio University |
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Principal Investigator |
MASHIMA Yukihiko School of Medicine, Keio University, Associate professor, 医学部, 助教授 (40157186)
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| Co-Investigator(Kenkyū-buntansha) |
KUDOH Jun School of Medicine, Keio University, Assistant professor, 医学部, 講師 (80178003)
OHTAKE Yuichirou School of Medicine, Keio University, Assistant professor, 医学部, 助手 (30233159)
KUROSAKA Daijirou School of Medicine, Keio University, Assistant professor, 医学部, 講師 (20215099)
IWATA Takeshi Tokyo Medical Center, Chief Investigator, 主任研究員
高柳 淳 慶應義塾大学, 医学部, 助手 (80245464)
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| Project Period (FY) |
2000 – 2001
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| Project Status |
Completed (Fiscal Year 2001)
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| Budget Amount *help |
¥15,500,000 (Direct Cost: ¥15,500,000)
Fiscal Year 2001: ¥7,500,000 (Direct Cost: ¥7,500,000)
Fiscal Year 2000: ¥8,000,000 (Direct Cost: ¥8,000,000)
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| Keywords | retinal ganglion cell / promotor / mause amine oxidase / Leber's disease / idebenone / human amine oxidase / ベクター |
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| Research Abstract |
Mouse homologue of AOC2 was cloned for haracterization. Real-time quantitative PCR and in situ hybridization were performed to determine specific expression of AOC2 in mouse retina, especially in retinal ganglion cells. The copper-binding motif and the enzyme active site of AOC1 and AOC3 were both preserved in mouse AOC2 protein. Mouse AOC2 gene was located 1,526 bp downstream of Psme3 gene for PA-28 gamma-subunit and 775 bp upstream of AOC3 gene. We have plan to construct the vector that can particularly transport some gene into ganglion cells.
Visual recovery and its characteristics were investigated in patients with Leber's hereditary optic neuropathy (LHON) developed in teens. Fourteen patients who had the 11778 mutation were included in this study. The averaged age of onset of the disease was 14.8 years. Six of 14 patients were treated with idebenone and vitamins B2 and C. Seven of the 14 LHON patients (11 of 28 eyes) showed a visual recovery between 0.3 and 1.0, and fenestrated central scotomas by the Humphrey 10-2 threshold. Five of the 7 patients were treated with medicines. The nasal side of the central visual fields showed a greater sensitivity than the temporal side in 10 of the 11 eyes. Nerve fiber bundles other than the papillomacular bundles may have a greater potential to recover function in patients with LHON.
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Report
(3 results)
Research Products
(6 results)
