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β-半乳糖苷酶-神经氨酸酶缺乏症:蛋白酶抑制剂恢复β-半乳糖苷酶活性

Beta-galactosidase-neuraminidase deficiency: restoration of beta-galactosidase activity by protease inhibitors.

作者信息

Suzuki Y, Sakuraba H, Hayashi K, Suzuki K, Imahori K

出版信息

J Biochem. 1981 Jul;90(1):271-3. doi: 10.1093/oxfordjournals.jbchem.a133462.

DOI:10.1093/oxfordjournals.jbchem.a133462
PMID:6793566
Abstract

Beta-Galactosidase was partially restored by protease inhibitors, leupeptin, chymostatin and E-64 in cultured fibroblasts from three patients with beta-galactosidase-neuraminidase deficiency. Pepstatin did not activate this enzyme. Neuraminidase was not affected by any of these compounds in the culture medium. It was concluded that the activating effect was produced by a specific inhibition of thiol proteases.

摘要

在来自三名β-半乳糖苷酶-神经氨酸酶缺乏症患者的培养成纤维细胞中,蛋白酶抑制剂、亮抑酶肽、抑肽酶和E-64可部分恢复β-半乳糖苷酶的活性。胃蛋白酶抑制剂不能激活这种酶。培养基中的任何一种化合物都不会影响神经氨酸酶。得出的结论是,激活作用是由硫醇蛋白酶的特异性抑制产生的。

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引用本文的文献

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