Maret A, Salvayre R, Nègre A, Douste-Blazy L
Biomedicine. 1980 May;33(3):82-6.
Electrofocusing allows to separate the beta-glucosidases from normal spleen in several molecular forms: a non specific beta-glucosidase form (pl 4.6) and two forms of beta-glucocerebrosidase (pl 5.0 and pl 6.5). beta-glucosidase and beta-glucocerbrosidase differ with regard to their thermal stability. In a spleen from a patient with non neuronopathis Gaucher's disease (type I or adult form), molecular whereas the non specific beta-glucosidase is not significantly decreased. Thus, the beta-glucocerebrosidase forms and the non specific beta-glucosidase are not coded by the same gene. The separation of molecular forms of beta-glucosidase allows to measure the part of specific beta-glucosidase activity in the assay of beta-glucocerebrosidase by artificial substrate, 4-methylumbeliferyl-beta-D-glucopyranoside.
等电聚焦可将正常脾脏中的β-葡萄糖苷酶分离为几种分子形式：一种非特异性β-葡萄糖苷酶形式（等电点4.6）和两种β-葡萄糖脑苷脂酶形式（等电点5.0和6.5）。β-葡萄糖苷酶和β-葡萄糖脑苷脂酶在热稳定性方面存在差异。在一名非神经元型戈谢病（I型或成人型）患者的脾脏中，分子形式的β-葡萄糖脑苷脂酶明显减少，而非特异性β-葡萄糖苷酶没有显著降低。因此，β-葡萄糖脑苷脂酶形式和非特异性β-葡萄糖苷酶并非由同一基因编码。β-葡萄糖苷酶分子形式的分离使得在通过人工底物4-甲基伞形酮基-β-D-吡喃葡萄糖苷测定β-葡萄糖脑苷脂酶时能够测量特异性β-葡萄糖苷酶活性的比例。
