Table II.
Activity of lysosomal enzymes in DUOC-01 cell products.
| Activity (nmol/h/mg protein) of 11 lysosomal enzymes in 5 cGMP lots of DUOC-01 | |||||||
|---|---|---|---|---|---|---|---|
|
| |||||||
| Enzyme | UCB No.
|
Mean | SD | ||||
| 600661 | 601558 | 601217 | 220058 | 220115 | |||
| β-Galactosidase | 914.4 | 902 | 281.2 | 1184.2 | 974 | 851.2 | 302.5 |
| β-Mannosidase | 72.8 | 75.8 | 54.9 | 98.7 | 66.2 | 73.7 | 14.4 |
| α-L-fucosidase | 1819 | 1661 | 5452 | 2171 | 3409 | 2902.4 | 1414.7 |
| α-Mannosidase | 503.8 | 574 | 377.5 | 636 | 757.6 | 569.8 | 127.3 |
| β-Glucuronidase | 1493 | 1620 | 943.7 | 1754 | 1429 | 1447.9 | 275.7 |
| β-Hexosaminidase A | 2351 | 2173 | 1647 | 3246 | 2684 | 2420.2 | 532.0 |
| Arylsulfatase A | 119.6 | 52.2 | 200.1 | 115 | 79.3 | 113.2 | 49.9 |
| Galactocerebrosidase | 1.5 | 6.9 | 14.3 | 9.46 | 6.51 | 7.7 | 4.2 |
| Sphingomyelinase | 16.4 | 22.6 | 30.1 | 27.1 | 20.6 | 23.4 | 4.8 |
| Glucocerebrosidase | 127 | 255.9 | 428.9 | 307 | 373.9 | 298.5 | 103.9 |
| α-L-iduronidase | 48.6 | 65.5 | 43.3 | 28.3 | 36.4 | 44.4 | 12.5 |
Analysis performed by Lysosomal Diseases Testing Laboratory, Thomas Jefferson University.