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Modification of subunit interaction in membrane‐bound acid β‐glucosidase from Gaucher disease
Author(s) -
Maret Arlette,
Potier Michel,
Salvayre Robert,
Douste-Blazy Louis
Publication year - 1983
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/0014-5793(83)80943-0
Subject(s) - protein subunit , chemistry , biochemistry , disease , medicine , gene
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.