title: alpha-D-mannosidase in the liver and brain from patients with I-cell disease.
abstract: The properties of alpha-D-mannosidase in the liver and brain from patients with I-cell disease were investigated, using 4-methylumbelliferyl-alpha-D-mannopyranoside as a substrate. A combination of chromatographies on DEAE-cellulose, concanavalin A-Sepharose and Sephacryl S-300 showed that intermediate alpha-D-mannosidase was present in the liver and brain from I-cell disease as well as control liver and brain, although an abnormal distribution of intermediate and acidic alpha-D-mannosidases occurred in the tissues from I-cell disease. These results suggested that a defect of intermediate alpha-D-mannosidase is unlikely to be the primary defect responsible for I-cell disease.
mesh_terms: D001921:Brain / Q000201:enzymology*; D002848:Chromatography, DEAE-Cellulose; D002850:Chromatography, Gel; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008099:Liver / Q000201:enzymology*; D008361:Mannosidases / Q000032:analysis*; D009081:Mucolipidoses / Q000201:enzymology*
chemical_list: D008361:Mannosidases