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Major characteristics of AB−/−, A−/− and B−/− mice
| AB−/− | A−/− | B−/− | |
|---|---|---|---|
| Life span (days) | 96 | 85 | 644 |
| Body weight (% WT) | 86 | 64 | 100 |
| Neuromotor deterioration | Rapid | More rapid | Slow |
| Brain pathology | Severe | Severe | Severe |
| Liver pathology | Severe | Severe | Normal |
| GSL accumulations | |||
| Sulfatide (brain and kidney) | High | Normal | High |
| GalCer (brain and kidney) | Normal | High | Normal |
| Galactosylsphingosine (brain) | Low | High | Normal |
| LacCer (liver) | High | High | Normal |
