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Literature summary extracted from

  • Tropak, M.B.; Kornhaber, G.J.; Rigat, B.A.; Maegawa, G.H.; Buttner, J.D.; Blanchard, J.E.; Murphy, C.; Tuske, S.J.; Coales, S.J.; Hamuro, Y.; Brown, E.D.; Mahuran, D.J.
    Identification of pharmacological chaperones for Gaucher disease and characterization of their effects on beta-glucocerebrosidase by hydrogen/deuterium exchange mass spectrometry (2008), ChemBioChem, 9, 2650-2662.
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
3.2.1.45 medicine inhibitors 5-((4-methylphenyl)thio)-quinazoline 2,4-diamine and 5-(3,5-dichlorophenoxy)-N-(4-pyridinyl)-2-furamide raise the levels of functional GCase 1.5–2.5-fold in N370S or F213I Gaucher disease fibroblasts. Treated fibroblasts from patients with Gaucher disease show decreased levels of enzyme in their endoplasmic reticulum and increased levels in lysosomes. Compound stabilizes a domain III active-site loop Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.2.1.45 F213I inhibitors 5-((4-methylphenyl)thio)-quinazoline 2,4-diamine and 5-(3,5-dichlorophenoxy)-N-(4-pyridinyl)-2-furamide raise the levels of functional GCase 1.5-2.5-fold in N370S or F213I Gaucher disease fibroblasts. Treated fibroblasts from patients with Gaucher disease show decreased levels of enzyme in their endoplasmic reticulum and increased levels in lysosomes. Compound stabilizes a domain III active-site loop Homo sapiens
3.2.1.45 N370S inhibitors 5-((4-methylphenyl)thio)-quinazoline 2,4-diamine and 5-(3,5-dichlorophenoxy)-N-(4-pyridinyl)-2-furamide raise the levels of functional GCase 1.5-2.5-fold in N370S or F213I Gaucher disease fibroblasts. Treated fibroblasts from patients with Gaucher disease show decreased levels of enzyme in their endoplasmic reticulum and increased levels in lysosomes. Compound stabilizes a domain III active-site loop Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.2.1.45 5-((4-methylphenyl)thio)-quinazoline 2,4-diamine inhibition of wild-type enzyme and chaperone function on mutant enzyme. Treated fibroblasts from patients with Gaucher disease show decreased levels of enzyme in their endoplasmic reticulum and increased levels in lysosomes. Compound stabilizes a domain III active-site loop Homo sapiens
3.2.1.45 5-(3,5-dichlorophenoxy)-N-(4-pyridinyl)-2-furamide inhibition of wild-type enzyme and chaperone function on mutant enzyme. Treated fibroblasts from patients with Gaucher disease show decreased levels of enzyme in their endoplasmic reticulum and increased levels in lysosomes. Compound stabilizes a domain III active-site loop Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.45 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 + glucose
-
 ?

IC50 Value

EC Number IC50 Value IC50 Value Maximum Comment Organism Inhibitor Structure
3.2.1.45 0.0047
-
 pH 5.5, 37°C Homo sapiens 5-(3,5-dichlorophenoxy)-N-(4-pyridinyl)-2-furamide
3.2.1.45 0.0078
-
 pH 5.5, 37°C Homo sapiens 5-((4-methylphenyl)thio)-quinazoline 2,4-diamine