Table 4.
α-Galactosidase activities in plasma and leukocytes from three known patients with Fabry diseasea
| Patient | Plasma (nmol/h per ml)
|
Leukocytes (nmol/h per mg protein)
|
||||
|---|---|---|---|---|---|---|
| Total | A | B | Total | A | B | |
| 1 | 0.6 | 0.0 | 0.6 | 12.6 | 6.9 | 5.7 |
| 2 | 3.6 | 3.6 | 0.0 | 9.3 | 5.2 | 4.1 |
| 3 | 8.0 | 7.2 | 0.7 | 3.9 | 2.3 | 1.7 |
a
Activities were assayed in the absence and presence of 100 mM GalNAc inhibitor (thus measuring “Total” and “A,” respectively). Reference range for total activity in leucocytes 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.