Modification of subunit interaction in membrane‐bound acid β‐glucosidase from Gaucher disease

Abstract

The radiation inactivation method has been used to determine the molecular mass of membrane‐bound acid β‐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 β‐glucosidase. From the results, we conclude that subunit interaction is altered in mutant acid β‐glucosidase and that this may be due to a direct effect of the mutation.