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人类细胞中溶酶体神经节苷脂唾液酸酶的鉴定。

The identification of lysosomal ganglioside sialidase in human cells.

作者信息

Zeigler M, Sury V, Bach G

机构信息

Department of Human Genetics, Hadassah University Hospital, Jerusalem, Israel.

出版信息

Eur J Biochem. 1989 Aug 1;183(2):455-8. doi: 10.1111/j.1432-1033.1989.tb14949.x.

DOI:10.1111/j.1432-1033.1989.tb14949.x
PMID:2759093
Abstract

In this report we present evidence for the existence of a lysosomal ganglioside sialidase. The sialidase activity was solubilized by sonication and stimulated by cholate. The absence of ganglioside sialidase activity in sialidosis patients indicates that lysosomal sialidase is active towards gangliosides and glycoproteins. The plasma membranes were associated with two types of ganglioside sialidase activities, one was enhanced by cholate while the other was partially inhibited by this detergent.

摘要

在本报告中,我们提供了溶酶体神经节苷脂唾液酸酶存在的证据。唾液酸酶活性通过超声处理溶解,并受胆酸盐刺激。唾液酸沉积症患者缺乏神经节苷脂唾液酸酶活性,这表明溶酶体唾液酸酶对神经节苷脂和糖蛋白具有活性。质膜与两种类型的神经节苷脂唾液酸酶活性相关,一种受胆酸盐增强,而另一种则被这种去污剂部分抑制。

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1
The identification of lysosomal ganglioside sialidase in human cells.人类细胞中溶酶体神经节苷脂唾液酸酶的鉴定。
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2
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Partial characterization and enrichment of a membrane-bound sialidase specific for gangliosides from human brain tissue.人脑组织中对神经节苷脂具有特异性的膜结合唾液酸酶的部分特性鉴定与富集
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Degradation of gangliosides by the lysosomal sialidase requires an activator protein.溶酶体唾液酸酶对神经节苷脂的降解需要一种激活蛋白。
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Further evidence that human lysosomal sialidase is not derived from prosaposin. Prosaposin biosynthesis and ganglioside sialidase studies in prosaposin- and sialidase-deficient fibroblast lines.进一步证明人溶酶体唾液酸酶并非源自前体唾液酸蛋白。前体唾液酸蛋白生物合成及前体唾液酸蛋白和唾液酸酶缺陷型成纤维细胞系中的神经节苷脂唾液酸酶研究。
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引用本文的文献

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Mucolipidosis type IV: abnormal transport of lipids to lysosomes.IV型黏脂贮积症:脂质向溶酶体的转运异常。
J Inherit Metab Dis. 1997 Sep;20(5):625-32. doi: 10.1023/a:1005362123443.
2
Freeze-stable sialidase activity in human leucocytes: substrate specificity, inhibitor susceptibility, detergent requirements and subcellular localization.人白细胞中冻融稳定的唾液酸酶活性:底物特异性、抑制剂敏感性、去污剂需求及亚细胞定位
Biochem J. 1994 Aug 1;301 ( Pt 3)(Pt 3):777-84. doi: 10.1042/bj3010777.
3
Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase.
一名患者及其患有鞘脂激活蛋白(SAP)前体—— prosaposin基因缺陷的胎儿同胞的其他生化检查结果。证明存在SAP-1缺乏以及溶酶体神经氨酸酶正常。
Biochem J. 1992 Jul 15;285 ( Pt 2)(Pt 2):481-8. doi: 10.1042/bj2850481.