Table 3.

Activities of α -galactosidase in leukocytes and plasma of five renal patients who had low plasma α-galactosidase activity in the initial screening phase of the studya

PatientLeukocytes (nmol/h per mg protein)Plasma (nmol/h per ml)
Initial Result (No GalNAc Inhibitor)Results on RecallInitial Result (No GalNAc Inhibitor)Results on Recall
TotalTotalATotalTotalA
166.751.051.31.62.31.5
2125.089.787.22.85.85.6
373.660.155.92.95.95.6
494.160.360.02.94.54.1
558.834.733.72.54.54.1
  • a In the initial phase, only total α-galactosidase activity was measured. When these patients were recalled for a second time, assays were performed both without and with 100 mM GalNAc inhibitor (thus measuring “Total” and “A” activity, respectively). Reference range for total activity in leukocytes 42.1 to 112.9 nmol/h per mg protein; reference range for total activity in plasma 3.0 to 8.1 nmol/h per ml.