Aerts J M, Donker-Koopman W E, van der Vliet M K, Jonsson L M, Ginns E I, Murray G J, Barranger J A, Tager J M, Schram A W
Eur J Biochem. 1985 Aug 1;150(3):565-74. doi: 10.1111/j.1432-1033.1985.tb09058.x.
The beta-glucosidase activity in spleen from control subjects and patients with different clinical phenotypes of Gaucher's disease was characterized. The occurrence of a soluble non-specific beta-glucosidase with a neutral pH optimum and two membrane-associated beta-glucocerebrosidases with an acid pH optimum was demonstrated. The two beta-glucocerebrosidases can be distinguished on the basis of their ability to react with anti-(placental beta-glucocerebrosidase) antibodies bound to protein-A--Sepharose 4B beads. One of the splenic beta-glucocerebrosidases (form I) is precipitated by the immobilized antibodies and the other (form II) is not. The two forms also differ in binding affinity to concanavalin A, degree of stimulation of enzymic activity by taurocholate and isoelectric point. In contrast, the Km values of the two beta-glucocerebrosidases for natural and artificial substrates are similar and both are inhibited by conduritol B-epoxide. In spleen from three patients with type 1, one patient with type 2 and one patient with type 3 Gaucher's disease form I beta-glucocerebrosidase was found to be clearly deficient, whereas the activity of form II was 25-50% of that in control spleen. The non-specific, neutral beta-glucosidase was not deficient in these Gaucher spleens. The distinct biochemical and immunological properties of non-specific beta-glucosidase and the fact that normal levels of the enzyme are present in patients with Gaucher's disease indicate, in confirmation of previous reports, that non-specific beta-glucosidase is not related to beta-glucocerebrosidase.
对正常受试者以及患有不同临床表型戈谢病患者脾脏中的β-葡萄糖苷酶活性进行了表征。结果表明，存在一种最适pH为中性的可溶性非特异性β-葡萄糖苷酶以及两种最适pH为酸性的膜相关β-葡萄糖脑苷脂酶。这两种β-葡萄糖脑苷脂酶可根据它们与结合在蛋白A-琼脂糖4B珠上的抗（胎盘β-葡萄糖脑苷脂酶）抗体反应的能力来区分。脾脏中的一种β-葡萄糖脑苷脂酶（I型）可被固定化抗体沉淀，而另一种（II型）则不能。这两种形式在与伴刀豆球蛋白A的结合亲和力、牛磺胆酸盐对酶活性的刺激程度以及等电点方面也存在差异。相比之下，这两种β-葡萄糖脑苷脂酶对天然和人工底物的Km值相似，且均受到 conduritol B-环氧化物的抑制。在3例1型、1例2型和1例3型戈谢病患者的脾脏中，发现I型β-葡萄糖脑苷脂酶明显缺乏，而II型的活性为正常脾脏的25%-50%。在这些戈谢病脾脏中，非特异性中性β-葡萄糖苷酶并不缺乏。非特异性β-葡萄糖苷酶独特的生化和免疫学特性以及戈谢病患者中该酶水平正常这一事实，证实了先前的报道，即非特异性β-葡萄糖苷酶与β-葡萄糖脑苷脂酶无关。
