Chabás A, Guardiola A, Burguera J M
Instituto de Bioquíquímica Clínica, Barcelona, Spain.
Biochem Int. 1987 Aug;15(2):449-57.
beta-Glucosidase-stimulating proteins have been purified from human brain. One of these proteins also activated oligosaccharide sialidase activity in fibroblasts from galactosialidosis and sialidosis patients and in control cells but was not able to stimulate residual sialidase from I-cell disease fibroblasts. Activation was observed with either sialyl-oligosaccharides and -glycoproteins or the artificial substrate MU-NANA. The activator did not stimulate ganglioside sialidase from control and mucolipidosis IV fibroblasts. Column chromatography, polyacrylamide electrophoresis or desialylation treatment of the activator did not achieve separation of the stimulating abilities toward beta-glucosidase and sialidase.
β-葡萄糖苷酶刺激蛋白已从人脑中纯化出来。其中一种蛋白还激活了来自半乳糖唾液酸贮积症和唾液酸贮积症患者以及对照细胞的成纤维细胞中的寡糖唾液酸酶活性，但不能刺激I型细胞病成纤维细胞中的残余唾液酸酶。用唾液酸化寡糖和糖蛋白或人工底物MU-NANA均可观察到激活作用。该激活剂不能刺激对照和黏脂贮积症IV型成纤维细胞中的神经节苷脂唾液酸酶。对该激活剂进行柱色谱、聚丙烯酰胺电泳或去唾液酸化处理，均未实现对β-葡萄糖苷酶和唾液酸酶刺激能力的分离。
