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  • Title: Modification of subunit interaction in membrane-bound acid beta-glucosidase from Gaucher disease.
    Author: Maret A, Potier M, Salvayre R, Douste-Blazy L.
    Journal: FEBS Lett; 1983 Aug 22; 160(1-2):93-7. PubMed ID: 6411492.
    Abstract:
    The radiation inactivation method has been used to determine the molecular mass of membrane-bound acid beta-glucosidase (EC 3.2.1.21) in situ, in normal human spleen and in that of two patients with type I Gaucher disease: the molecular mass in Gaucher spleen is about double (125 000 +/- 8900) of that found in the normal spleen (67 000 +/- 7700) which is compatible with the existence of subunit coupling in the muted acid beta-glucosidase. From the results, we conclude that subunit interaction is altered in mutant acid beta-glucosidase and that this may be due to a direct effect of the mutation.
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