Immunological Studies on Lysosomal Sphingomyelinase: Immunization Procedures, Properties of Polyclonal and Monoclonal Antibodies Obtained and Effect of Triton X-100 on Binding of Enzyme Activity
Immunological Studies on Lysosomal Sphingomyelinase: Immunization Procedures, Properties of Polyclonal and Monoclonal Antibodies Obtained and Effect of Triton X-100 on Binding of Enzyme Activity
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溶酶体鞘磷脂酶的免疫学研究：免疫程序、获得的多克隆和单克隆抗体的特性以及 Triton X-100 对酶活性结合的影响
DOI：
10.1007/978-1-4684-5212-9_39
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发表时间：
1986
期刊：
影响因子：
--
通讯作者：
A. Schram
中科院分区：
文献类型：
--
作者：
E. Al;G. Weitz;K. Sandhoff;J. Barranger;J. Hilgers;J. Tager;A. Schram
鞘磷脂酶将鞘磷脂水解成神经酰胺和磷酰胆碱。在患有 A 型和 B 型尼曼匹克病的患者的组织中，这种溶酶体膜相关酶缺乏，并且鞘磷脂积聚1。在 C 型尼曼-匹克病患者的组织中，鞘磷脂也会积聚，但鞘磷脂酶并不缺乏1。
Sphingomyelinase hydrolyses sphingomyelin to ceramide and phosphoryl choline. In tissues from patients suffering from Niemann-Pick disease type A and B this lysosomal, membrane-associated enzyme is deficient and sphingomyelin accumulates1. In tissues from patients with Niemann-Pick disease type C, sphingomyelin also accumulates but sphingomyelinase is not deficient1.