| Project/Area Number |
01440044
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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 |
Pediatrics
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| Research Institution | Tokyo Jikei University School of Medicine |
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Principal Investigator |
MAEKAWA K. Jikei Univ. Pediatrics Professor, 医学部, 教授 (80056613)
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| Co-Investigator(Kenkyū-buntansha) |
奈良 隆寛 東京慈恵会医科大学, 医学部, 講師 (80180537)
TOKORO T. Jikei Univ. Pediatrics assistant prof., 医学部, 講師 (40112841)
ITO F. Jikei Univ. Pediatrics assoc. prof., 医学部, 助教授 (10057010)
ETO Y. Jikei Univ. Pediatrics assoc. prof., 医学部, 助教授 (50056909)
SAKURAI S. Jikei Univ. Molecular Lab. Professor, 医学部, 教授 (20056542)
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| Project Period (FY) |
1989 – 1991
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| Project Status |
Completed (Fiscal Year 1991)
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| Budget Amount *help |
¥14,200,000 (Direct Cost: ¥14,200,000)
Fiscal Year 1991: ¥2,400,000 (Direct Cost: ¥2,400,000)
Fiscal Year 1990: ¥2,900,000 (Direct Cost: ¥2,900,000)
Fiscal Year 1989: ¥8,900,000 (Direct Cost: ¥8,900,000)
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| Keywords | Krabbe Disease / Nimann-Pick Disease / Psychosine / Signal Transduction / NGF receptor / golgiーlysosomal complex / NGFRcDNA / NGF / NGFRカスケ-ド / NiemannーPick病マウス / twitcherマウス / スフィンゴミエリナ-ゼ / Blood Brain barrier / マンノ-ス含有オリゴ糖 / リポゾ-ム療法 / 動物モデル / Niemann-Pichマウス / レトロウイルスベクタ- |
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| Research Abstract |
1) Pathogenesis and treatment using animal model of Krabbe's disease : Animal inodel of Krabbe disease, named twitcher's brain was cultured and chased morphological and biochemical changes in oligodendroglial cell cultures. Pyschosine was accumulated 100 times more than control in twitcher tissues. the accumulation of psychosine alters membrane signal transduction system. 2) Niemann-Pick mouse model : Niemann-Pick mouse in mouse is equivalent to human Niemann-Pick type C, since free cholesterol and various glycolipids were accumulated in this mutant mice. The accumulation of these lipids are closely related to Golgi-lysosomal membrane complexes. Therefore, we speculate that abnormality of this mutant mice may be caused by lysosomal membrane transport system. 3) Introduction of nerve growth factor gene into neuroblastma cells. We succeeded transfection of NGF receptor gene intoneuroblastma cells and found benign process of neural cells.
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