Mercelis R, Van Elsen A F, Leroy J G
Clin Chim Acta. 1979 Apr 2;93(1):85-92. doi: 10.1016/0009-8981(79)90247-x.
A new technique is introduced for the differential assay of arylsulphatases A and B in centrifuged homogenates of cultured human skin fibroblasts, using 4-methylumbelliferyl-sulphate as a substrate and AgNO3 as a selective inhibitor of arylsulphatase A. The method can be applied in the diagnosis of metachromatic leucodystrophy, mucopolysaccharidosis type VI and mucosulphatidosis. Normal arylsulphatase activities were found in fibroblasts derived from patients with mucopolysaccharidoses types II, III-A and IV, known to be caused by deficiencies of various other sulphatases.
介绍了一种新技术,用于在培养的人皮肤成纤维细胞的离心匀浆中对芳基硫酸酯酶A和B进行差异测定,该技术使用4-甲基伞形酮基硫酸盐作为底物,硝酸银作为芳基硫酸酯酶A的选择性抑制剂。该方法可用于异染性脑白质营养不良、VI型黏多糖贮积症和黏脂贮积症的诊断。在已知由其他各种硫酸酯酶缺乏引起的II型、III-A型和IV型黏多糖贮积症患者的成纤维细胞中发现了正常的芳基硫酸酯酶活性。