Modifications of the molecular weight of membrane-bound nonspecific beta-glucosidase in type 1 Gaucher disease determined in situ by the radiation inactivation method.

The radiation inactivation method has been used to compare the molecular weight of the nonspecific membrane-bound beta-glucosidase in situ in normal human spleen and in that of two patients with Gaucher disease type 1. We report, in type 1 Gaucher spleen, the presence of a high molecular weight component (557 000) in addition to the normal low molecular weight component (97 800). The various possible hypotheses explaining this high molecular weight component are discussed.