Sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate as activators of spleen glucocerebrosidase.
Sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate as activators of spleen glucocerebrosidase.
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磺基半乳糖脑苷脂和双-(单酰基甘油)-磷酸作为脾葡萄糖脑苷脂酶的激活剂。
DOI：
10.1016/0009-8981(86)90151-8
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发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Huterer,S
中科院分区：
文献类型：
--
作者：
Prence,EM;Garrett,KO;Panitch,H;Basu,A;Glew,RH;Wherrett,JR;Huterer,S
用胆酸钠和正丁醇连续提取人脾膜去除内源性脂质并使葡糖脑苷脂酶活性依赖于外源性酸性脂质（例如，磷脂酰丝氨酸、神经节苷脂）和热稳定激活蛋白（HSF）。在本报告中，我们表明，两个以前未经测试的溶酶体酸性脂质，即磺基半乳糖苷和双-此外，磺基半乳糖苷还显著增强了从1型糖尿病患者中分离的葡萄糖脑苷脂酶的活性。（非神经病性）戈谢病，导致比活性为对照葡糖脑苷脂酶的60-80%。此外，当硫脂被用作激活剂时，来自1型患者的葡萄糖脑苷脂酶的活性是来自2型患者的相应葡萄糖脑苷脂酶的30倍相反，两种BMP，一种富含C26饱和脂肪酸，另一种富含C18不饱和脂肪酸，是两种突变型葡糖脑苷脂酶的相对较差的活化剂，同时提供对照活性的优异重建。
Sequential extraction of human spleen membranes with sodium cholate andn-butanol removes endogenous lipids and renders glucocerebrosidase activity dependent upon exogenous acidic lipids (e.g., phosphatidylserine, gangliosides) and a heat-stable activator protein (HSF). In the present report, we show that two previously untested lysosomal acidic lipids, namely sulfogalactocerebroside and bis-(monoacylglyceryl)-phosphate (BMP), also activate normal human glucocerebrosidase.In addition, sulfogalactocerebroside also markedly enhanced the activity of glucocerebrosidase isolated from a patient with type 1 (non-neuronopathic) Gaucher's disease, resulting in a specific activity which was 60–80% that of control glucocerebrosidase. Furthermore, when the sulfolipid was used as the activator, glucocerebrosidase from the type 1 patient was 30 times more active than the corresponding glucocerebrosidase from a person with type 2 (neuronopathic) Gaucher's disease.In contrast, the two BMPs, one rich in C26saturated fatty acid and another rich in C18unsaturated fatty acids, were relatively poor activators of both mutant glucocerebrosidases while providing excellent reconstitution of control activity.