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正常儿童和成胱氨酸尿症儿童成纤维细胞中酸性水解酶的活性。

Activities of acid hydrolases in fibroblasts from normal and cystinotic children.

作者信息

Scardigli K, Koldovsky O, Palmieri M, Segal S, States B

出版信息

Clin Chim Acta. 1977 Feb 15;75(1):13-20. doi: 10.1016/0009-8981(77)90495-8.

Abstract

A study of the lysosomal hydrolases bete-galactosidase, beta-glucuronidase, N-acetyl-beta-D-glucosaminidase, and arylsulphatases A and B has been carried out on fibroblasts cultured from seven patients with cystinosis and eight control subjects. beta-Galactosidase activity was found to be consistently lower in cells derived from cystinotics, while the other enzymes studied showed no significant differences between normals and cystinotics.

摘要

对7名胱氨酸病患者和8名对照受试者培养的成纤维细胞进行了溶酶体水解酶β-半乳糖苷酶、β-葡萄糖醛酸酶、N-乙酰-β-D-氨基葡萄糖苷酶以及芳基硫酸酯酶A和B的研究。发现胱氨酸病患者来源的细胞中β-半乳糖苷酶活性一直较低,而所研究的其他酶在正常人和胱氨酸病患者之间未显示出显著差异。

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