EC Number   |
Activating Compound |
Reference |
|---|
    3.2.1.22 | 1-deoxygalactonojirimycin |
3.8fold increase at 0.02 mM after 5 days |
678069 |
| 3.2.1.22 | 1-deoxygalactonojirimycin |
acts as a chemical chaperone and enhances catalytic activity and protein expression in milder mutations associated with the atypical form of Fabry disease |
697800 |
| 3.2.1.22 | 1-deoxygalactonojirimycin |
T-cells from normal controls resond with 28% increase in alpha-galactosidase activity to treatment with 1-deoxygalactonojirimycin. In cells from patients with Fabry disease, response depends on he mutantion and ranges from no increase to fully normal activity. In normal cells and in cells from patients responding more than 25%, an increase in the mature lysosomal form of enzyme is observed after treatment. In the group of intermediate respoders with increases of 7-25% in activity upon treatment, an increase in protein stain but incomplete processing of the enzyme to the mature form is detected |
700602 |
| 3.2.1.22 | 2-mercaptoethanol |
107.8% activity at 10 mM |
680270 |
| 3.2.1.22 | 2-mercaptoethanol |
required for enzymatic activity |
714250 |
| 3.2.1.22 | 6-deoxy-D-glucose |
the enzyme is produced exclusively in the presence of the specific inducer 6-deoxy-D-glucose, also named quinovose |
680975 |
| 3.2.1.22 | beta-mercaptoethanol |
activity enhanced by 2fold |
713881 |
| 3.2.1.22 | carbodiimide |
165% activity at 0.4 mM |
749609 |
| 3.2.1.22 | D-glucose |
105.1% activity at 10 mM |
680270 |
| 3.2.1.22 | D-glucose |
increase of activity |
171199 |
