Clinical appearance and β-glucuronidase expression in mucopolysaccharidosis VII mice transplanted in utero with AA4.1-enriched, cultured transgenic fetal liver cells
| Age (days) . | Feature . | Mouse 1 . | Mouse 2 . | Mouse 3 . | Mouse 4 . |
|---|---|---|---|---|---|
| 60 | Mobility | good | good | fair-poor | fair-poor |
| Facial appearance | pointed | pointed | blunted | blunted | |
| Urinary GAGs | +/− | +/− | +++ | +++ | |
| GUSB activity (toe clip) | 14% | 18% | 0% | 0% | |
| 214 | Mobility | very poor | very poor | very poor | very poor |
| Facial appearance | blunted | blunted | blunted | blunted | |
| Urinary GAGs | +++ | +++ | +++ | +++ | |
| GUSB activity | 0% | 0% | 0% | 0% | |
| GUSB-positive cells | Spleen, Liver | spleen, liver | none | none | |
| H-2Dd-positive cells | Spleen, liver, brain | spleen, liver, brain | spleen | none |
| Age (days) . | Feature . | Mouse 1 . | Mouse 2 . | Mouse 3 . | Mouse 4 . |
|---|---|---|---|---|---|
| 60 | Mobility | good | good | fair-poor | fair-poor |
| Facial appearance | pointed | pointed | blunted | blunted | |
| Urinary GAGs | +/− | +/− | +++ | +++ | |
| GUSB activity (toe clip) | 14% | 18% | 0% | 0% | |
| 214 | Mobility | very poor | very poor | very poor | very poor |
| Facial appearance | blunted | blunted | blunted | blunted | |
| Urinary GAGs | +++ | +++ | +++ | +++ | |
| GUSB activity | 0% | 0% | 0% | 0% | |
| GUSB-positive cells | Spleen, Liver | spleen, liver | none | none | |
| H-2Dd-positive cells | Spleen, liver, brain | spleen, liver, brain | spleen | none |
GAGs indicates glycosaminoglycans; GUSB, β-glucuronidase.