1988 Fiscal Year Final Research Report Summary
Study for the mechanism of Neurological Manifestation in lysosomal storage disorders
| Project/Area Number |
61570476
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Nihon University School of Medicine |
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Principal Investigator |
SAKIYAMA T. Assistant Professor, 医学部小児科, 講師 (20130510)
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| Co-Investigator(Kenkyū-buntansha) |
WATANABE C. Instructor, 医学部小児科, 助手
AKATSUKA A. Instructor, 医学部小児科, 助手 (60183133)
OWADA M. Assistant Professor, 医学部小児科, 講師 (40059506)
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| Project Period (FY) |
1986 – 1988
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| Keywords | Niemann-Pick disease / Niemann-Pick mouse / Nitrogen cavitation / lysosomal enzyme / コレステロール |
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| Research Abstract |
The Niemann-Pick mice have been considered a useful model for the neuropathic form (type A or C) of Niemann-Pick disease in himans. Firstly, using this animal model, we showed a remarkably reduced activity of acid sphingomyelinase in the brain lysosomal fraction as well as liver by the method of sucrose gradient subfractionation. The accumulated lipids were also noticed in the fractionated part of the affected brain. In addition, the morphological changes in lyosomes were noticed by electronmicroscopical findings. Secondary, nitrogen cavitation and Percoll gradient method, which is a new technique for studying intracellular fractionation,seems to be a useful tool to study small amounts of tissur or cultured cell. The fractionated pattern of sphingomyelinase activities showed the similar results from the sucrose gradient subfractionation. Finally, Niemann-Pick mice (C57BL/KsJ spm/spm) were fed with water containing 0.25% dimetyl sulfoxide (DMSO), then their life spans and time course of body weight were dhecked. DMSO treated spm/spm mice lived slightly longer than non-treated mice. However, the mechanism how DMSO prolong the life span is still unclear.
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