Accumulation of GM2 Ganglioside in Niemann-Pick Disease Type C Fibroblasts.
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- YANO Tamami
- Department of Neurobiology, School of Life Sciences, Tottori University Faculty of Medicine
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- TANIGUCHI Miyako
- Department of Neurobiology, School of Life Sciences, Tottori University Faculty of Medicine
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- AKABOSHI Shinjiro
- Division of Child Neurology, Institute of Neurological Sciences, Tottori University Faculty of Medicine
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- VANIER Marie-Thèrése
- Department of Biochemistry, INSERM-CNRS 189, Lyon-Sud School of Medicine
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- TAI Tadashi
- Departments of Tumor Immunology and Clinical Genetics, Tokyo Metropolitan Institute of Medical Scinece
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- SAKURABA Hitoshi
- Departments of Tumor Immunology and Clinical Genetics, Tokyo Metropolitan Institute of Medical Scinece
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- OHNO Kousaku
- Department of Neurobiology, School of Life Sciences, Tottori University Faculty of Medicine
Bibliographic Information
- Other Title
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- Accumulation of GM2 Ganglioside in Niem
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Abstract
Niemann-Pick disease type C (NP-C) is an autosomal recessive neurovisceral lipid storage disorder biochemically characterized by a defect in intracellular transport of low-density lipoprotein (LDL)-derived cholesterol from the lysosome to other cellular sites. We have found substantial accumulation of GM2 ganglioside in NP-C fibroblasts. The intracellular distribution of GM2 ganglioside was similar to that of cholesterol detected by filipin staining, indicating that the accumulation of GM2 ganglioside is mainly lysosomal. The incorporation of N-acetyl-D-[3H]mannosamine into gangliosides was also increased in NP-C fibroblasts, especially into the GM2 and GM3 fractions. A culture condition which eliminates cholesterol accumulation does not eliminate GM2 accumulation. It is suggested that the accumulation of GM2 ganglioside together with the accumulation of cholesterol is a unique abnormality in NP-C fibroblasts and that the defect in NP-C may involve intracellular transport of both cholesterol and GM2 ganglioside.
Journal
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- Proceedings of the Japan Academy, Series B
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Proceedings of the Japan Academy, Series B 72 (10), 214-219, 1996
The Japan Academy
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Details 詳細情報について
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- CRID
- 1390282679122194432
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- NII Article ID
- 130000903761
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- NII Book ID
- AA00785485
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- ISSN
- 13492896
- 03862208
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- NDL BIB ID
- 4176386
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- Text Lang
- en
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- Data Source
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- JaLC
- NDL
- Crossref
- CiNii Articles
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- Abstract License Flag
- Disallowed