| Project/Area Number |
09671788
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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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 | Tokyo Medical and Dental University |
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Principal Investigator |
INABA Akira (1999) Tokyo Medical and Dental University, Department of Medical Laboratory, Research Associate, 医学部, 助手 (10282766)
横田 隆徳 (1997-1998) 東京医科歯科大学, 医学部, 助手 (90231688)
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| Co-Investigator(Kenkyū-buntansha) |
MIZUSAWA Hidehiro Tokyo Medical and Dental University, Department of Neurology, Professor, 医学部, 教授 (30144091)
INOUE Keizo University of Tokyo, Department of Health Chemistry, Professor, 薬学部, 教授 (30072937)
UCHIHARA Toshiki Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Research Associate, 東京都神経科学総合研究所・神経病理学部門, 主任研究員 (10223570)
SAITO Yukinobu Tokyo Medical and Dental University, Department of Neurology, Research Associate, 医学部, 助手 (80302842)
KIYOSAWA Motohiro Tokyo Medical and Dental University, Department of Ophthalmology, Assistant Professor, 医学部, 助教授 (20169957)
稲葉 彰 東京医科歯科大学, 医学部, 助手 (10282766)
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| Project Period (FY) |
1997 – 1999
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| Project Status |
Completed (Fiscal Year 1999)
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| Budget Amount *help |
¥3,800,000 (Direct Cost: ¥3,800,000)
Fiscal Year 1999: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1998: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 1997: ¥2,400,000 (Direct Cost: ¥2,400,000)
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| Keywords | α-tocopherol / knockout mouse / vitamin E / retinitis pigmentosa / spinocerebellar degeneration / Bergmann glia / posterior column / Purkinje cell / α-トコフェロール / αTTP / Friedriech失調症 / 脊髄小脳変異症 |
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| Research Abstract |
1) Alpha-tocopherol transfer protein knockout mouse
We finished making mouse in which alpha-tocopherol transfer protein gene was deleted. Now, we are investigating pathology of central nervous system, retina, and their functions using somatosensory evoked potentials and retinograms.
2) Distribution of alpha-tocopherol transfer protein
We identify the expression of alpha-tocopherol transfer protein in retina, cerebellum, spinal cord by Northern blot. Furthermore in situ hybridization technique demonstrated that the local expression of the protein in the Bergmann glia in cerebellum. There was no expression in dorsal root ganglia, or peripheral nervous system.
3) Investigation on autopsy of patient with mutant alpha-tocopherol transfer protein gene
The major pathological findings were retinal atrophy ; severe degeneration of the dying back-type in the posterior column ; and massive accumulation of ceroid-lipofuscin in neurons including dorsal root ganglion (DRG) cells. In addition, mild loss of Purkinje cells was noted. In the DRG, thought to be mainly responsible for ataxia, no expression of alpha-tocopherol transfer protein was detected, and the tissue concentration of vitamin E increased to normal after supplementation. We therefore concluded that oral supplementation of vitamin E should effectively counteract the progression of ataxia.
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