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Literature summary extracted from

  • Steet, R.A.; Chung, S.; Wustman, B.; Powe, A.; Do, H.; Kornfeld, S.A.
    The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms (2006), Proc. Natl. Acad. Sci. USA, 103, 13813-13818.
    View publication on PubMedView publication on EuropePMC

Activating Compound

EC Number Activating Compound Comment Organism Structure
3.2.1.45 isofagomine GlcCerase activity is enhanced in normal fibroblasts 1.3fold after treatment with 0.03 mM isofagomine for 5 days Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.2.1.45 N370S decreased activity Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.2.1.45 isofagomine
-
 Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.2.1.45 lysosome
-
 Homo sapiens 5764
-

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.45 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.45 CCD-43Sk cell
-
 Homo sapiens
-
3.2.1.45 fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.45 4-methylumbelliferyl-beta-D-glucopyranoside + H2O
-
 Homo sapiens methylumbelliferone + beta-D-glucose
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.2.1.45 GlcCerase
-
 Homo sapiens

IC50 Value

EC Number IC50 Value IC50 Value Maximum Comment Organism Inhibitor Structure
3.2.1.45 0.000005
-
 wild type enzyme, at pH 7.2 Homo sapiens isofagomine
3.2.1.45 0.000009
-
 wild type enzyme, at pH 6.4 Homo sapiens isofagomine
3.2.1.45 0.000018
-
 mutant enzyme N370S, at pH 5.2 Homo sapiens isofagomine
3.2.1.45 0.000026
-
 mutant enzyme N370S, at pH 6.4 Homo sapiens isofagomine
3.2.1.45 0.00003
-
 wild type enzyme, at pH 5.2 Homo sapiens isofagomine
3.2.1.45 0.000128
-
 mutant enzyme N370S, at pH 7.2 Homo sapiens isofagomine