Analysis of storage glycosphingolipids GM2 and GA2 at the terminal stage of disease in Sandhoff mice
| Tissue . | Storage GSLs (% untreated control) . | Therapeutic group . | ||
|---|---|---|---|---|
| SHNB-DNJ . | SHBMT . | SHBMT/NB-DNJ . | ||
| Brain | GM2 | 107.2 ± 10.4 | 104.6 ± 15.3 | 133.0 ± 18.7* |
| GA2 | 96.3 ± 7.8 | 94.7 ± 7.1 | 103.4 ± 1.5 | |
| Spinal cord | GM2 | 111.1 ± 3.1 | 98.3 ± 12.1 | 134.7 ± 18.1* |
| GA2 | 107.4 ± 4.1 | 79.9 ± 5.2* | 88.3 ± 8.1* | |
| Liver | GM2 | 82.7 ± 0.5* | 43.6 ± 4.4* | 40.1 ± 4.8* |
| GA2 | 104.7 ± 7.3 | ND | ND | |
| Tissue . | Storage GSLs (% untreated control) . | Therapeutic group . | ||
|---|---|---|---|---|
| SHNB-DNJ . | SHBMT . | SHBMT/NB-DNJ . | ||
| Brain | GM2 | 107.2 ± 10.4 | 104.6 ± 15.3 | 133.0 ± 18.7* |
| GA2 | 96.3 ± 7.8 | 94.7 ± 7.1 | 103.4 ± 1.5 | |
| Spinal cord | GM2 | 111.1 ± 3.1 | 98.3 ± 12.1 | 134.7 ± 18.1* |
| GA2 | 107.4 ± 4.1 | 79.9 ± 5.2* | 88.3 ± 8.1* | |
| Liver | GM2 | 82.7 ± 0.5* | 43.6 ± 4.4* | 40.1 ± 4.8* |
| GA2 | 104.7 ± 7.3 | ND | ND | |
Data are representative of 3 independent analyses of the same samples (n = 4-6 per group); ND corresponds to levels that are undetectable by high performance thin layer chromatography.
Significantly different from the control untreated values (see text for details).