Table 3.
Organ | Group (no. of mice) | α-gal A activity, nmol/hr/ mg protein | Gb3, nmol/mg protein |
---|---|---|---|
Liver | Wild type: 12 weeks | ||
Untouched (5) | 69 ± 4 | 0.1 ± 0.1 | |
Knockout: 12 weeks | |||
Untouched (4) | 0.7 ± 0.1 | 3.1 ± 1.1 | |
1,100 rads–mock (5) | 1.2 ± 0.3 | 7.5 ± 1.2 | |
550 rads–transduced (5) | 20 ± 12* | 0.1 ± 0.2† | |
1,100 rads–transduced (6) | 19 ± 6 | 0.1 ± 0.0† | |
Knockout: 26 weeks | |||
1,100 rads–transduced (6) | 27 ± 20* | 0.1 ± 0.0† | |
Spleen | Wild type: 12 weeks | ||
Untouched (5) | 453 ± 50 | 0.1 ± 0.1 | |
Knockout: 12 weeks | |||
Untouched (4) | 9 ± 1 | 15 ± 4 | |
1,100 rads–mock (5) | 18 ± 2 | 15 ± 1.2 | |
550 rads–transduced (5) | 390 ± 190* | 1.1 ± 0.9† | |
1,100 rads–transduced (6) | 210 ± 96† | 1.4 ± 0.3† | |
Knockout: 26 weeks | |||
1,100 rads–transduced (6) | 300 ± 220* | 3.7 ± 1.4* | |
Heart | Wild type: 12 weeks | ||
Untouched (5) | 10 ± 1.3 | 0.2 ± 0.1 | |
Knockout: 12 weeks | |||
Untouched (4) | 0.2 ± 0.1 | 1.1 ± 0.2 | |
1,100 rads–mock (5) | 0.3 ± 0.1 | 2.1 ± 0.5 | |
550 rads–transduced (5) | 2.0 ± 1.1* | 0.4 ± 0.2† | |
1,100 rads–transduced (6) | 3.3 ± 1.2† | 0.3 ± 0.1† | |
Knockout: 26 weeks | |||
1,100 rads–transduced (6) | 4.2 ± 3.3* | 0.9 ± 0.3* | |
Lung | Wild type: 12 weeks | ||
Untouched (5) | 81 ± 5 | 0.5 ± 0.1 | |
Knockout: 12 weeks | |||
Untouched (4) | 1.5 ± 0.5 | 5.3 ± 0.3 | |
1,100 rads–mock (5) | 1.8 ± 0.2 | 4.2 ± 0.2 | |
550 rads–transduced (5) | 14 ± 8* | 2.7 ± 0.9* | |
1,100 rads–transduced (6) | 9.2 ± 2.7† | 1.9 ± 0.5† | |
Knockout: 26 weeks | |||
1,100 rads–transduced (6) | 15 ± 15 | 1.7 ± 1.0† | |
Kidney | Wild type: 12 weeks | ||
Untouched (5) | 48 ± 6 | 1.4 ± 7.4 | |
Knockout: 12 weeks | |||
Untouched (4) | 1.0 ± 0.1 | 8.7 ± 2.3 | |
1,100 rads–mock (5) | 1.1 ± 0.1 | 10.7 ± 1.4 | |
550 rads–transduced (5) | 2.2 ± 0.6* | 7.3 ± 0.7† | |
1,100 rads–transduced (6) | 3.0 ± 1.5* | 8.0 ± 1.9* | |
Knockout: 26 weeks | |||
1,100 rads–transduced (6) | 3.1 ± 1.3* | 2.9 ± 1.7† | |
Brain | Wild type: 12 weeks | ||
Untouched (5) | 130 ± 3 | 0.2 ± 0.1 | |
Knockout: 12 weeks | |||
Untouched (4) | 1.2 ± 0.4 | 0.3 ± 0.1 | |
1,100 rads–mock (5) | 1.4 ± 0.2 | 0.4 ± 0.0 | |
550 rads–transduced (5) | 2 ± 0 | 0.3 ± 0.1 | |
1,100 rads–transduced (6) | 2 ± 0 | 0.2 ± 0.1 | |
Knockout: 26 weeks | |||
1,100 rads–transduced (6) | 0.8 ± 0.2 | 0.2 ± 0.1 |
*, P < 0.05; †, P < 0.01 vs. 1,100 rads–mock group.