Table 2. Specific Activities for Lysosomal Enzymes Using the New and Original Assay Reagentsa.
| original reagent |
new reagent |
|||||
|---|---|---|---|---|---|---|
| enzyme | spec. act. (μmol/h/L) | spec. act. no blood (μmol/h/L) | ratio of complete assay to no-blood assay response | spec. act. (μmol/h/L) | spec. act. no blood (μmol/h/L) | ratio blood/no blood |
| MPS-I | 1.57 | 0.03 | 52.3 | 1.21 | 0.004 | 293.5 |
| MPS-II | 2.88 | 0.076 | 37.9 | 4.40 | 0.06 | 75 |
| MPS-VI (anion exch., EtOAc) | 1.41 | 0.28 | 4.91 | 12.17 | 0.125 | 97.4 |
| MPS-VI (EtOAc) | 1.15 | 0.24 | 4.75 | 13.125 | 0.79 | 16.7 |
a
All specific activities are not blank-corrected.