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  • 标题:Lipid abnormalities in foam cell reticulosis of mice, an analogue of human sphingomyelin lipidosis
  • 本地全文:下载
  • 作者:Donald P. Fredrickson ; Howard R. Sloan ; Carl T. Hansen
  • 期刊名称:JLR Papers In Press
  • 印刷版ISSN:0022-2275
  • 电子版ISSN:1539-7262
  • 出版年度:1969
  • 卷号:10
  • 期号:3
  • 页码:288-293
  • 语种:English
  • 出版社:American Society for Biochemistry and Molecular Biology
  • 摘要:The lipid changes in the inheritable foam cell reticulosis of mice discovered by Lyons, Hulse, and Rowe have been reexamined. The major abnormality in thymuses from homozygous-abnormal animals has been identified as an increase in the concentration (per milligram of protein) of sphingomyelin and cholesterol. This increase is associated with normal sphingomyelin-cleaving activity. The lipid compositions of the liver and spleen in the homozygous-abnormal animal and of the thymus in the heterozygous-abnormal mouse are normal. The disorder appears to be chemically analogous to those forms of human sphingomyelin lipidosis (Niemann-Pick disease) that are not accompanied by a decrease in tissue sphingomyelinase.
  • 关键词:inherited ; lipid-storage disease ; sphingomyelin ; cholesterol ; thymus ; sphingomyelin-cleaving enzyme ; lysolecithin ; Niemann-Pick disease
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