Svennerholm L, Håkansson G, Månsson J E, Vanier M T
Clin Chim Acta. 1979 Feb 15;92(1):53-64. doi: 10.1016/0009-8981(79)90396-6.
Methods were elaborated for the assay on leukocytes of sphingolipid hydrolases with native and artificial substrates. The optimal conditions for the isolation of leukocytes and the preparation of labelled sphingolipids were investigated. The KM and Vmax values for all substrates are given as the specific activities of the sphingolipidhydrolases in normal males and females. It is concluded that leukocytes are the enzyme source of choice for the enzymic diagnosis of neurolipidoses.
阐述了使用天然和人工底物测定白细胞中鞘脂水解酶的方法。研究了分离白细胞和制备标记鞘脂的最佳条件。给出了所有底物的米氏常数(KM)和最大反应速度(Vmax)值,以正常男性和女性中鞘脂水解酶的比活性表示。得出结论,白细胞是神经脂质沉积症酶学诊断的首选酶源。