Hultberg B, Sjöblad S, Ockerman P A
Acta Paediatr Scand. 1975 Jan;64(1):123-31.
Five glycosidases, alpha-fucosidase, alpha-galactosidase, alpha-glucosidase, beta-mannosidase and N-acetyl-alpha-glucosaminidase were studied in human skin fibroblast cultures. The pH-dependency, kinetic properties of the enzymes and results of isoelectric focusing and ion exchange chromatography are presented. Techniques suitable for diagnosing inborn lysosomal diseases in skin fibroblast cultures are defined.
在人皮肤成纤维细胞培养物中研究了五种糖苷酶，即α-岩藻糖苷酶、α-半乳糖苷酶、α-葡萄糖苷酶、β-甘露糖苷酶和N-乙酰-α-葡糖胺糖苷酶。介绍了这些酶的pH依赖性、动力学特性以及等电聚焦和离子交换色谱分析结果。确定了适用于诊断皮肤成纤维细胞培养物中先天性溶酶体疾病的技术。
