Isoelectric focusing of acid hydrolases in human liver and serum. Findings in sera from one patient with I-cell disease phenotype.

Isoelectric focusing was performed with six acid hydrolases in serum and liver tissue. Neuraminidase-treated serum and liver tissue were also examined. In general, acid hydrolases in sera seemed to be more sialylated than those in liver tissue. Serum from one patient with I-cell disease with increased activity of some acid hydrolases was found to have a normal isoelectric focusing pattern. These findings are discussed with respect to the uptake mechanism of acid hydrolases in serum.