alpha-D-mannosidases in serum of patients with I-cell disease (ICD).
The specific activity of alpha-D-mannosidase in serum of ICD-patients is considerably increased due to increased amounts of the component with optimal activity at pH 4.6 (acidic form). The intermediate form with pH optimum of 6.0 remains unaltered. These conclusions were reached by using optimal conditions for differential assay of the alpha-D-mannosidases checked by partial separation of the components in serum by sucrose gradient centrifugation.
D004355:Drug Stability; D006358:Hot Temperature; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008361:Mannosidases / Q000097:blood*; D009081:Mucolipidoses / Q000201:enzymology*
D008361:Mannosidases