1998 Fiscal Year Final Research Report Summary
Mechanism of cerebellar neuronal cell death in CTX patients
| 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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| Keywords | CTX / cerebellar neuronal cells / apoptosis / cholestanol / corneal endothelial cells / lens epithelial cells |
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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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[Publications] Chen, W., Kubota, S.Teramoto, T., Nishimura, Y., Yonemoto, K., and Seyama, Y.: "Silent nucleotide substitution in the sterol 27 hydroxylase gene (CYP27) leads to alternative pre-mRNA splicing by activating a cryptic 5' splice site at the mutant codon in cerebrotendinous xanthomatosis patients." Biochemistry. 37. 4420-4428 (1998)
Description
「研究成果報告書概要(欧文)」より
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