β-半乳糖苷酶-α-神经氨酸酶缺乏症中β-半乳糖苷酶的诱导：亮抑酶肽和蔗糖的作用。

Induction of beta-galactosidase in beta-galactosidase-alpha-neuraminidase deficiency: effects of leupeptin and sucrose.

作者信息

Kato T, Okada S, Yutaka T, Yabuuchi H

出版信息

Biochem Int. 1983 Feb;6(2):267-73.

Abstract

beta-Galactosidase was normalized by a serine-thiol protease inhibitor, leupeptin with concentration of 10 micrograms/ml in cultured skin fibroblasts from patients with beta-galactosidase-alpha-neuraminidase deficiency (beta-Gal-/Neu-). The induction of this enzyme was not observed in normal cells. Because the enzymic activity of cathepsin B1 increased significantly both in beta-Gal-/Neu- and normal cells by leupeptin loading, the restoration of beta-galactosidase in beta-Gal-/Neu- cells can not be explained by the theory that leupeptin inhibited intracellular degradation of beta-galactosidase molecules. The effects of leupeptin and sucrose on lysosomal hydrolase induction were compared.

摘要

在来自β-半乳糖苷酶-α-神经氨酸酶缺乏症（β-Gal-/Neu-）患者的培养皮肤成纤维细胞中，β-半乳糖苷酶用浓度为10微克/毫升的丝氨酸-硫醇蛋白酶抑制剂亮肽素进行标准化。在正常细胞中未观察到这种酶的诱导。由于亮肽素加载后，组织蛋白酶B1的酶活性在β-Gal-/Neu-细胞和正常细胞中均显著增加，因此β-Gal-/Neu-细胞中β-半乳糖苷酶的恢复不能用亮肽素抑制β-半乳糖苷酶分子细胞内降解的理论来解释。比较了亮肽素和蔗糖对溶酶体水解酶诱导的影响。

相似文献

1

Induction of beta-galactosidase in beta-galactosidase-alpha-neuraminidase deficiency: effects of leupeptin and sucrose.β-半乳糖苷酶-α-神经氨酸酶缺乏症中β-半乳糖苷酶的诱导：亮抑酶肽和蔗糖的作用。

Biochem Int. 1983 Feb;6(2):267-73.

2

Immunoelectron microscopical localization of lysosomal beta-galactosidase and its precursor forms in normal and mutant human fibroblasts.溶酶体β-半乳糖苷酶及其前体形式在正常和突变型人成纤维细胞中的免疫电子显微镜定位

Eur J Cell Biol. 1986 Mar;40(1):9-15.

3

Neuraminidase deficiency and accumulation of sialic acid in lymphocytes in adult type sialidosis with partial beta-galactosidase deficiency.成人型唾液酸沉积症伴部分β-半乳糖苷酶缺乏时的神经氨酸酶缺乏及淋巴细胞中唾液酸的蓄积

Ann Neurol. 1982 May;11(5):541-3. doi: 10.1002/ana.410110517.

4

Galactosialidosis (beta-galactosidase-neuraminidase deficiency): a possible role of serine-thiol proteases in the degradation of beta-galactosidase molecules.半乳糖唾液酸贮积症（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

Clin Chim Acta. 1982 Nov 10;125(3):275-82. doi: 10.1016/0009-8981(82)90257-1.

5

Involvement of thiol proteases in galactosialidosis.硫醇蛋白酶与半乳糖唾液酸贮积症的关系。

Clin Chim Acta. 1986 Mar 16;155(2):109-15. doi: 10.1016/0009-8981(86)90271-8.

6

Complementation, cross correction, and drug correction studies of combined beta-galactosidase neuraminidase deficiency in human fibroblasts.人类成纤维细胞中β-半乳糖苷酶神经氨酸酶联合缺乏的互补、交叉校正及药物校正研究。

Pediatr Res. 1984 Feb;18(2):167-71. doi: 10.1203/00006450-198402000-00011.

7

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

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

8

beta-Galactosidase and neuraminidase deficiency associated with angiokeratoma corporis diffusum.

Arch Dermatol. 1984 Oct;120(10):1344-6.

9

Galatosialidosis (beta-galactosidase-neuraminidase deficiency): clinical and biochemical studies on 13 patients.

Adv Exp Med Biol. 1982;152:241-51.

10

Adult mucolipidosis with beta-galactosidase and neuraminidase deficiencies.伴有β-半乳糖苷酶和神经氨酸酶缺乏的成人粘脂贮积症

J Neurol Sci. 1980 May;46(2):245-54. doi: 10.1016/0022-510x(80)90083-0.

引用本文的文献

1

The effects of sucrose loading on lysosomal hydrolases.蔗糖负荷对溶酶体水解酶的影响。

Mol Cell Biochem. 1984;60(1):83-98. doi: 10.1007/BF00226302.

2

Effects of the protease inhibitor leupeptin on proteolytic activities and regeneration of mouse skeletal muscles after exercise injuries.蛋白酶抑制剂亮抑蛋白酶肽对运动损伤后小鼠骨骼肌蛋白水解活性及再生的影响。

Am J Pathol. 1984 Oct;117(1):64-70.

文献AI研究员

20分钟写一篇综述，助力文献阅读效率提升50倍。

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

文档翻译

学术文献翻译模型，支持多种主流文档格式。