Modification of subunit interaction in membrane-bound acid beta-glucosidase from Gaucher disease.
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.
D002462:Cell Membrane / Q000201:enzymology*; D005776:Gaucher Disease / Q000201:enzymology*; D005959:Glucosidases / Q000378:metabolism*; D006801:Humans; D006867:Hydrolases / Q000378:metabolism; D007700:Kinetics; D046911:Macromolecular Substances; D008970:Molecular Weight; D012016:Reference Values; D013154:Spleen / Q000201:enzymology*; D001617:beta-Glucosidase / Q000378:metabolism* / Q000528:radiation effects
D046911:Macromolecular Substances; D006867:Hydrolases; D005959:Glucosidases; D001617:beta-Glucosidase