Table 2.
The effect of variable core structure with constant n-nonyl substructure on N370S and WT β-Glu activity
| Compound | Relative maximum N370S ICA increase (concentration) | IC50 N370S | IC50 WT |
|---|---|---|---|
| 1 NN-DNJ | 1.65 (5 μM) | 5 μM | 1 μM |
| 2 | 1.10 (20 μM) | 330 μM | 100 μM |
| 3 | 1.40 (30 μM) | 540 μM | 175 μM |
| 4 | 1.50 (5 μM) | 450 μM | 110 μM |
| 5 | No activity | >10 mM | >10 mM |
| 6 | Slightly inhibitory | >1 mM | >1 mM |
 |
Various core structures N-alkylated with the n-nonyl group were added to the culture medium of fibroblasts for 5 days. β-Glu activity was assayed at pH 4.0 by using 4-methyllumbelliferyl β-d glucoside as a substrate. IC50 values were determined in WT and N370S lysates at pH 5.0 in the presence of 0.1% Triton X-100 and 0.2% taurodeoxycholic acid. Enzyme activity is normalized to untreated cells, assigned a relative activity of 1. ICA, intact cell β-Glu activity.