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  • Title: [Separation by electrofocusing of the molecular forms of splenic beta-glucosidase in normal subjects and Gaucher's disease].
    Author: Maret A, Salvayre R, Nègre A, Douste-Blazy L.
    Journal: Biomedicine; 1980 May; 33(3):82-6. PubMed ID: 6778520.
    Abstract:
    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.
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