Lysosomal enzyme activities of various organs and platelets of normal C57BL/6J, single mutant (ep/ep andpe/pe), and double mutant (ep/ep,pe/pe) mice
| . | β-Glucuronidase (U/g) . | β-Galactosidase (U/g) . |
|---|---|---|
| Kidney | ||
| C57BL/6J (8) | 61.4 ± 4.5 | 14.8 ± .64 |
| ep/ep(6) | 143.0 ± 6.53-150 | 27.2 ± 1.13-150 |
| pe/pe(7) | 145.0 ± 5.03-150 | 19.2 ± 0.73-150 |
| ep/ep, pe/pe(8) | 229.0 ± 15.03-150 | 37.6 ± 2.83-150 |
| Lung | ||
| C57BL/6J | 4.73 ± 0.12 | 3.29 ± 0.14 |
| ep/ep | 5.20 ± 0.26 | 3.64 ± 0.18 |
| pe/pe | 6.08 ± 0.79 | 3.65 ± 0.28 |
| ep/ep,pe/pe | 12.2 ± 1.23-150 | 6.27 ± 0.593-150 |
| Liver | ||
| C57BL/6J | 25.4 ± 1.8 | 9.02 ± 0.79 |
| ep/ep | 27.7 ± 1.9 | 10.0 ± 0.80 |
| pe/pe | 23.7 ± 2.3 | 11.7 ± 0.58 |
| ep/ep, pe/pe | 29.0 ± 0.8 | 10.0 ± 1.1 |
| Brain | ||
| C57BL/6J | 0.84 ± 0.02 | 3.13 ± 0.11 |
| ep/ep | 0.96 ± 0.06 | 2.88 ± 0.06 |
| pe/pe | 0.96 ± 0.05 | 3.11 ± 0.07 |
| ep/ep,pe/pe | 1.06 ± 0.033-150 | 2.88 ± 0.14 |
| Spleen | ||
| C57BL/6J | 16.0 ± 0.77 | 17.5 ± 1.2 |
| ep/ep | 18.4 ± 2.3 | 18.2 ± 2.5 |
| pe/pe | 22.0 ± 1.53-150 | 19.3 ± 0.84 |
| ep/ep,pe/pe | 21.5 ± 2.83-150 | 20.0 ± 1.4 |
| Platelets | ||
| C57BL/6J | .0516 ± .0012 | .0693 ± .0044 |
| ep/ep | .0587 ± .0025 | .0885 ± .0029 |
| pe/pe | .0491 ± .0043 | .0723 ± .0088 |
| ep/ep,pe/pe | .0501 ± .0024 | .0796 ± .0075 |
| . | β-Glucuronidase (U/g) . | β-Galactosidase (U/g) . |
|---|---|---|
| Kidney | ||
| C57BL/6J (8) | 61.4 ± 4.5 | 14.8 ± .64 |
| ep/ep(6) | 143.0 ± 6.53-150 | 27.2 ± 1.13-150 |
| pe/pe(7) | 145.0 ± 5.03-150 | 19.2 ± 0.73-150 |
| ep/ep, pe/pe(8) | 229.0 ± 15.03-150 | 37.6 ± 2.83-150 |
| Lung | ||
| C57BL/6J | 4.73 ± 0.12 | 3.29 ± 0.14 |
| ep/ep | 5.20 ± 0.26 | 3.64 ± 0.18 |
| pe/pe | 6.08 ± 0.79 | 3.65 ± 0.28 |
| ep/ep,pe/pe | 12.2 ± 1.23-150 | 6.27 ± 0.593-150 |
| Liver | ||
| C57BL/6J | 25.4 ± 1.8 | 9.02 ± 0.79 |
| ep/ep | 27.7 ± 1.9 | 10.0 ± 0.80 |
| pe/pe | 23.7 ± 2.3 | 11.7 ± 0.58 |
| ep/ep, pe/pe | 29.0 ± 0.8 | 10.0 ± 1.1 |
| Brain | ||
| C57BL/6J | 0.84 ± 0.02 | 3.13 ± 0.11 |
| ep/ep | 0.96 ± 0.06 | 2.88 ± 0.06 |
| pe/pe | 0.96 ± 0.05 | 3.11 ± 0.07 |
| ep/ep,pe/pe | 1.06 ± 0.033-150 | 2.88 ± 0.14 |
| Spleen | ||
| C57BL/6J | 16.0 ± 0.77 | 17.5 ± 1.2 |
| ep/ep | 18.4 ± 2.3 | 18.2 ± 2.5 |
| pe/pe | 22.0 ± 1.53-150 | 19.3 ± 0.84 |
| ep/ep,pe/pe | 21.5 ± 2.83-150 | 20.0 ± 1.4 |
| Platelets | ||
| C57BL/6J | .0516 ± .0012 | .0693 ± .0044 |
| ep/ep | .0587 ± .0025 | .0885 ± .0029 |
| pe/pe | .0491 ± .0043 | .0723 ± .0088 |
| ep/ep,pe/pe | .0501 ± .0024 | .0796 ± .0075 |
Values are the mean ± SEM of the number of mice indicated in parentheses (all organs). Platelet values are U/109platelets and are from assays on platelets from 4 separate mice in each case.
Significant increase; see text for P values.