title: Modifications of the molecular weight of membrane-bound nonspecific beta-glucosidase in type 1 Gaucher disease determined in situ by the radiation inactivation method.
abstract: 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.
mesh_terms: D000328:Adult; D005776:Gaucher Disease / Q000201:enzymology*; D005959:Glucosidases / Q000032:analysis*; D006596:Hexosaminidases / Q000032:analysis; D006801:Humans; D008566:Membranes / Q000201:enzymology; D008970:Molecular Weight; D013108:Sphingomyelin Phosphodiesterase / Q000032:analysis; D013154:Spleen / Q000201:enzymology; D001617:beta-Glucosidase / Q000032:analysis*; D001619:beta-N-Acetylhexosaminidases
chemical_list: D013108:Sphingomyelin Phosphodiesterase; D005959:Glucosidases; D006596:Hexosaminidases; D001617:beta-Glucosidase; D001619:beta-N-Acetylhexosaminidases