Stimulation of GM3 ganglioside sialidase activity by an activator protein in patients with mucolipidosis IV and controls.

Enzyme Pub Date : 1991-01-01 DOI:10.1159/000468861
Y Ben-Yoseph, D A Mitchell, R M Yager, R K Pretzlaff
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引用次数: 1

Abstract

An activator protein that stimulates the enzymic hydrolysis of sialic acid from gangliosides by ganglioside sialidase was fractionated from human liver. This fraction was distinct from those stimulating the hydrolysis of galactose from GM1 ganglioside by beta-galactosidase and the hydrolysis of N-acetylgalactosamine from GM2 ganglioside by hexosaminidase A. This fraction was highly specific for the hydrolysis of sialic acid from GM3 ganglioside, and was equally effective in fibroblasts from patients with mucolipidosis IV and in fibroblasts from controls.

激活蛋白对IV型粘脂病患者及对照组GM3神经节脂苷唾液酸酶活性的刺激作用。
从人肝脏中分离出一种激活蛋白,它能刺激神经节苷脂酶水解唾液酸。该部分不同于刺激β -半乳糖苷酶水解GM1神经节苷脂的半乳糖和己糖苷酶a水解GM2神经节苷脂的n -乙酰半乳糖胺。该部分对GM3神经节苷脂水解唾液酸具有高度特异性,对黏脂质沉积症IV患者的成纤维细胞和对照组的成纤维细胞同样有效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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