.ALPHA.-D-Mannosidase in the liver and brain from patients with I-cell disease.

MINAMI, R. and NAKAO, T. α-D-Mannosidase in the Liver and Brain from Patients with I-Cell Disease. Tohoku J. exp. Med., 1982, 136 (1), 43-51-The properties of α-D-mannosidase in the liver and brain from patients with I-cell disease were investigated, using 4-methylumbelliferyl-α-D-mannopyranoside as a substrate. A combination of chromatographies on DEAE-cellulose, concanavalin A-Sepharose and Sephacryl S-300 showed that intermediate α-D-mannosidae 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 ac idic α-D-mannosidases occurred in the tissues from I-cell disease. These results suggested that a defect of intermediate α-D-mannosidase is unlikely to be the primary defect responsible for I-cell disease.-intermediate α