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 - Suppr

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超能文献

Galactosialidosis (beta-galactosidase-neuraminidase deficiency): a possible role of serine-thiol proteases in the degradation of beta-galactosidase molecules.

作者信息

Sakuraba H, Aoyagi T, Suzuki Y

出版信息

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

DOI:10.1016/0009-8981(82)90257-1

PMID:6816485

Abstract

摘要

相似文献

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.

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

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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。

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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.

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

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Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man.人类β-半乳糖苷酶和神经氨酸酶联合缺乏的分子缺陷

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beta-Galactosidase-neuraminidase deficiency (galactosialidosis): clinical, pathological, and enzymatic studies in a postmortem case.

Ann Neurol. 1983 May;13(5):497-503. doi: 10.1002/ana.410130505.

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。

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beta-Galactosidase--neuraminidase deficiency. Deficiency of a freeze-labile neuraminidase in leukocytes and fibroblasts.β-半乳糖苷酶-神经氨酸酶缺乏症。白细胞和成纤维细胞中一种对冷冻敏感的神经氨酸酶缺乏。

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Biochem J. 1996 Feb 1;313 ( Pt 3)(Pt 3):787-94. doi: 10.1042/bj3130787.

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Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis.β-半乳糖苷酶缺陷小鼠作为GM1神经节苷脂贮积症的动物模型。

Glycoconj J. 1997 Sep;14(6):729-36. doi: 10.1023/a:1018573518127.

Protective protein gene mutations in galactosialidosis.半乳糖唾液酸贮积症中的保护蛋白基因突变。

J Clin Invest. 1993 Jun;91(6):2393-8. doi: 10.1172/JCI116472.

Intracellular processing and maturation of mutant gene products in hereditary beta-galactosidase deficiency (beta-galactosidosis).遗传性β-半乳糖苷酶缺乏症（β-半乳糖苷沉积症）中突变基因产物的细胞内加工与成熟

Hum Genet. 1994 Feb;93(2):109-14. doi: 10.1007/BF00210592.

GM1-Gangliosidosis: a molecular abnormality of acid beta-galactosidase in fibroblasts.GM1神经节苷脂贮积症：成纤维细胞中酸性β-半乳糖苷酶的分子异常。

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Adrenoleukodystrophy and beta-galactosidase deficiency: patient and carrier.肾上腺脑白质营养不良与β-半乳糖苷酶缺乏症：患者及携带者

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