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Literature summary extracted from

  • Steet, R.; Chung, S.; Lee, W.S.; Pine, C.W.; Do, H.; Kornfeld, S.
    Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase (2007), Biochem. Pharmacol., 73, 1376-1383.
    View publication on PubMedView publication on EuropePMC

Protein Variants

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

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.2.1.45 isofagomine
-
 Homo sapiens
3.2.1.45 N-butyldeoxynojirimycin
-
 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 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 acid-beta-glucosidase
-
 Homo sapiens
3.2.1.45 GlcCerase
-
 Homo sapiens

IC50 Value

EC Number IC50 Value IC50 Value Maximum Comment Organism Inhibitor Structure
3.2.1.45 0.002
-
 in 50 mM citrate buffer with 0.25% (v/v) Triton X-100 (pH 4.5) Homo sapiens N-butyldeoxynojirimycin
3.2.1.45 0.2
-
 in 50 mM citrate buffer with 0.25% (v/v) Triton X-100 (pH 4.5) Homo sapiens isofagomine