Karpova E A, Dudukina T V, Tsvetkova I V
Institute of Biological and Medical Chemistry, USSR Academy of Medical Sciences, Moscow.
Biochem Int. 1991 Aug;24(6):1135-44.
The following glycosides of 4-trifluoromethylumbelliferone: alpha-D-mannopyranoside, alpha-L-fucopyranoside, alpha-D-glucopyranoside, beta-D-glucopyranoside, alpha-D-galactopyranoside, beta-D-galactopyranoside, alpha-L-iduronide and beta-D-glucuronide were studied. 4-Trifluoromethylumbelliferyl glycosides were shown to be substrates for glycosidases. Some of them were cleaved even better than the corresponding methylumbelliferyl glycosides. 4-Trifluoromethylumbelliferyl glycosides were applied for revealing the corresponding enzyme deficiencies upon diagnosis of Gaucher and Hurler diseases as well as GM1 gangliosidosis and alpha-mannosidosis. 4-Trifluoromethylumbelliferone released after enzymatic hydrolysis of 4-trifluoromethylumbelliferyl glycosides exhibits more contrast yellow fluorescence in UV-light than the blue one of methylumbelliferone upon exposure of enzyme activity on solid supports. Therefore 4-trifluoromethylumbelliferyl glycosides are convenient substrates for revealing glycosidase activity directly in tissue samples, e.g. in placenta, and thus for fast prenatal diagnosis of lysosomal diseases.
对4-三氟甲基伞形酮的以下糖苷进行了研究：α-D-甘露吡喃糖苷、α-L-岩藻吡喃糖苷、α-D-葡萄糖吡喃糖苷、β-D-葡萄糖吡喃糖苷、α-D-半乳糖吡喃糖苷、β-D-半乳糖吡喃糖苷、α-L-艾杜糖醛酸苷和β-D-葡萄糖醛酸苷。4-三氟甲基伞形酮基糖苷被证明是糖苷酶的底物。其中一些糖苷的水解效果甚至比相应的甲基伞形酮基糖苷更好。4-三氟甲基伞形酮基糖苷用于在诊断戈谢病和胡勒病以及GM1神经节苷脂病和α-甘露糖苷病时揭示相应的酶缺陷。在固体支持物上酶活性作用下，4-三氟甲基伞形酮基糖苷酶促水解后释放的4-三氟甲基伞形酮在紫外线下呈现出比甲基伞形酮的蓝色荧光更明显的黄色荧光。因此，4-三氟甲基伞形酮基糖苷是直接在组织样本（如胎盘）中揭示糖苷酶活性的便捷底物，从而用于溶酶体疾病的快速产前诊断。
