DEFICIENCY OF SPHINGOMYELIN-CLEAVING ENZYME ACTIVITY IN TISSUE CULTURES DERIVED FROM PATIENTS WITH NIEMANN-PICK DISEASE

被引:88
作者
SLOAN, HR
UHLENDORF, BW
KANFER, JN
BRADY, RO
FREDRICKSON, DS
机构
[1] Molecular Disease Branch, National Heart Institute, Bethesda
[2] Laboratory of Viral Immunology, Division of Biologics Standards, Bethesda
[3] Laboratory of Neurochemistry, National Institute of Neurological Diseases and Blindness, Bethesda
关键词
D O I
10.1016/0006-291X(69)90777-3
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The activity of the enzyme which catalyzes the hydrolysis of sphingomyelin has been assayed in tissue cultures derived from the skin and bone marrow of patients with different forms of Niemann-Pick disease. Compared with the values obtained with control cells, the activity of this enzyme was markedly reduced in two forms of Niemann-Pick disease. © 1969.
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页码:582 / +
页数:1
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