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Literature summary for 3.2.1.45 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

Protein Variants Comment Organism
N370S increased activity Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
isofagomine
-
Homo sapiens
N-butyldeoxynojirimycin
-
Homo sapiens

Localization

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
acid-beta-glucosidase
-
Homo sapiens
GlcCerase
-
Homo sapiens

IC50 Value

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