| Project/Area Number |
09470039
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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 |
Pathological medical chemistry
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| Research Institution | The University of Tokyo |
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Principal Investigator |
SEYAMA Yousuke INSTITUTION,Graduate School of Medicine, The University of Tokyo, Professor, 大学院・医学系研究科, 教授 (90010082)
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| Co-Investigator(Kenkyū-buntansha) |
UCHIJIMA Yasunobu Graduate School of Medicine, The University of Tokyo, Assistant Professor, 大学院・医学系研究科, 助手 (90272426)
KANO Kazutaka Graduate School of Medicine, The University of Tokyo, Assistant Professor, 大学院・医学系研究科, 助手 (70111507)
KUBOTA Shunichiro Graduate School of Medicine, The University of Tokyo, Associate Professor, 大学院・医学系研究科, 助教授 (00260480)
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| Project Period (FY) |
1997 – 1998
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| Project Status |
Completed (Fiscal Year 1998)
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| Budget Amount *help |
¥13,100,000 (Direct Cost: ¥13,100,000)
Fiscal Year 1998: ¥4,800,000 (Direct Cost: ¥4,800,000)
Fiscal Year 1997: ¥8,300,000 (Direct Cost: ¥8,300,000)
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| Keywords | CTX / cerebellar neuronal cells / apoptosis / cholestanol / corneal endothelial cells / lens epithelial cells / 能研黄色腫症 / CTX |
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| Research Abstract |
CTX is a hereditary lipid storage disease characterized by hypercholestanolemia, cerebellar ataxia, Achilles tendon xanthoma, cataract, and premature atherosclerosis. The etiology of cerebellar ataxia and cataract is unknown. To clarify the etiology of cerebellar ataxia and cataract, we established model rats for CTX fed diets containing cholestanol. By histochemical analysis cholestanol was deposited in lens, cornea, and cerebellum. Therefore, we hypothesized that cholestanol may induce apoptosis of cerebellar neuronal cells including Purkinje cells, corneal endothelial cells, and lens epithelial cells. Primary culture of these cells were performed and cholestanol was added into culture media. Cholestanol induced apoptosis of these cells as judged by TUNEL method, induction of caspases, and morphological changes of these cells. The results in the present study may explain the mechanism of cerebellar ataxia and cataract in CTX patients.
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