Table 1.
Lysosomal enzyme activities in leukocytes, liver and spleen of the MPS VI affected dog (Proband), one clinically healthy dog (Control), and normal reference range (Ref.).
| Tissue | Arylsulphatase B (MPS VI)a | α-L-Iduronidase (MPS I) | β-D-Glucuronidase (MPS VII) | ||||||
|---|---|---|---|---|---|---|---|---|---|
| Proband | Control | Ref.b | Proband | Control | Ref. | Proband | Control | Ref. | |
| Leukocytes | 1.9c | 40.5 | 45.0±14.4 | 21.2 | 19.3 | 18.7±3.9 | 281.9 | 304.1 | 314.5±88.6 |
| Liver | 1.1 | 23.3 | 26.3±4.5 | 28.8 | 27.7 | 30.6±10.2 | 301.2 | 310.2 | 342.4±90.2 |
| Spleen | 1.4 | 42.1 | 45.0±18.4 | 27.1 | 24.6 | 22.2±9.0 | 408.2 | 426.5 | 400.0±105.3 |
a
Disease corresponds to the specific dysfunction of the enzyme tested
b
Reference ranges are based on 10 clinically healthy dogs, and presented as mean ± standard deviation
c
Enzyme activity: nMol/mg protein/hour