Differences in intracellular corrective activity in cultured mannosidosis cells of acidic alpha-mannosidases from...

The ability of acidic alpha-mannosidases from pig kidney, bovine liver, human urine and placenta, and jack bean to correct the deranged metabolism of cultured skin fibroblasts from manosidosis patients is studied. One cause for the different corrective abilities are, as shown for other systems, the different rates of endocytosis of the enzyme forms into the cells. Besides this, it is demonstrated for the first time, that there are differences in the intracellular corrective activity of the internalized enzymes, which can be explained by different specificities of the enzymes against the storage material.