| Project/Area Number |
62440044
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| Research Category |
Grant-in-Aid for General Scientific Research (A)
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| Allocation Type | Single-year Grants |
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| Research Field |
Dermatology
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| Research Institution | University of Tokyo |
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Principal Investigator |
ISHIBASHI Yasumasa Tokyo Univ. Medicine, Prof., 医学部・附属病院, 教授 (10010191)
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| Co-Investigator(Kenkyū-buntansha) |
WATANABE Ryoji Tokyo Univ. Medicine, Lecturer, 医学部・附属病院・分院, 構師 (80158675)
TSUCHIDA Tetsuya Tokyo Univ. Medicine, Lecturer, 医学部・附属病院, 講師 (70126126)
OTSUKA Fujio Tokyo Univ. Medicine, A. Prof., 医学部・附属病院・分院, 助教授 (10092157)
UMEDA Takashi Teikyo Univ. Medicine, Prof., 医学部・附属病院, 教授 (50107504)
ONODERA Kazukiyo Tokyo Univ. Agriculture, Prof., 農学部, 教授 (90012773)
盛岡 奈緒子 東京大学, 医学部, 助手 (80200393)
中川 秀己 東京大学, 医学部(病), 講師 (20114580)
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| Project Period (FY) |
1987 – 1990
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| Project Status |
Completed (Fiscal Year 1990)
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| Budget Amount *help |
¥17,300,000 (Direct Cost: ¥17,300,000)
Fiscal Year 1990: ¥300,000 (Direct Cost: ¥300,000)
Fiscal Year 1989: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 1988: ¥4,000,000 (Direct Cost: ¥4,000,000)
Fiscal Year 1987: ¥12,000,000 (Direct Cost: ¥12,000,000)
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| Keywords | neurocutaneous syndrome / Pringle's disease / tuberous sclerosis / Recklinghausen's disease / neurofibromatosis / 糖脂質 / 増殖因子 / 分裂異常 / モノクローナル抗体 / 神経線維腫 / 悪性神経鞘腫 / 血管線維腫 / シュワン細胞 / 細胞骨格蛋白 |
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| Research Abstract |
This reseach project aims to clarify the etiology and pathogenesis of tuberous sclerosis and neurofibromatosis, two major diseases of neurocutaneous syndrome.
Cultured cells derived from angiofibroma of tuberous sclerosis have been demonstrated to divide in abnormal fashion. This abnormality further revealed to result from the genetic control deterioration in intermediate filament formation. The abnormal gene has been found to locate on chromosome 6. These cellular and molecular abnormalities may well explain the phacomatotic nature of tuberous sclerosis and help to know its etiology.
Neurofibromas and neurofibrosarcomas were analyzed in terms of ganglioside composition in patients with neurofibromatosis. The ganglioside composition has been demonstrated to be characteristic for the respective tumors, which enables to distinguish one from the other. Constitutive cells in neurofibromas are examined and clarified. Clinical manifestations are also characterized in neurofibromatosis patients.
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