Galactosialidosis (β-galactosidase-neuraminidase deficiency): a possible role of serine-thiol proteases in the degradation of β-galactosidase molecules
Galactosialidosis (β-galactosidase-neuraminidase deficiency): a possible role of serine-thiol proteases in the degradation of β-galactosidase molecules
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半乳糖唾液酸沉积症（β-半乳糖苷酶神经氨酸酶缺乏症）：丝氨酸硫醇蛋白酶在β-半乳糖苷酶分子降解中的可能作用
DOI：
10.1016/0009-8981(82)90257-1
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发表时间：
1982
影响因子：
5
通讯作者：
Suzuki Yoshiyuki
中科院分区：
文献类型：
--
作者：
Sakuraba Hitoshi;Aoyagi Takaaki;Suzuki Yoshiyuki
用丝氨酸巯基蛋白酶抑制剂恢复培养的半乳糖唾液酸沉积症患者皮肤成纤维细胞中的β-半乳糖苷酶。神经氨酸酶不受这些化合物的影响。与对照细胞相比，患者成纤维细胞中的组织蛋白酶B活性中度增加。组织蛋白酶B等丝氨酸巯基蛋白酶可能参与了β-半乳糖苷酶的降解。
β-Galactosidase was restored by serine-thiol protease inhibitors in cultured skin fibroblasts from patients with galactosialidosis. Neuraminidase was not influenced by these compounds. Cathepsin B activity in patients' fibroblasts was moderately increased as compared to that in control cells. Some serine-thiol protease like cathepsin B may play a role in the degradation of β-galactosidase molecules in tissues of patients with this disease.