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3.2.1.45: glucosylceramidase

This is an abbreviated version!
For detailed information about glucosylceramidase, go to the full flat file.

Word Map on EC 3.2.1.45

Reaction

a D-glucosyl-N-acylsphingosine
+
H2O
=
D-glucose
 +
a ceramide

Synonyms

ABG, acid beta-D-glucosidase, acid beta-glucocerebrosidase, acid beta-glucosidase, acid beta-glucosylceramidase, acid beta-glycosidase, acid glucosylceramidase, acid-beta-glucocerebrosidase, acid-beta-glucosidase, Alglucerase, beta-D-glucocerebrosidase, beta-GC, beta-glucocerebrosidase, beta-glucosidase, beta-glucosidase 2, beta-glucosylceramidase, beta-glucosylceramidase 2, beta-glycosylceramidase, bile acid beta-glucosidase, ceramidase, glucosyl-, ceramide glucosidase, cerebroside beta-glucosidase, Ceredase, Cerezyme, D-glucosyl-N-acylsphingosine glucohydrolase, GBA, GBA1, GBA2, Gcase, GCD, GCR, GDA, gene-activated human GlcCerase, Glc-Cerase, glcCer-beta-glucosidase, GlcCerase, glucocerebrosidase, glucocerebroside beta-glucosidase, glucose cerebrosidase, glucosphingosine glucosylhydrolase, glucosylceramidase, glucosylceramide beta-glucosidase, glucosylcerebrosidase, glucosylsphingosine beta-D-glucosidase, glucosylsphingosine beta-glucosidase, glycosylceramidase, glycosylceramide-beta-glucosidase, Imiglucerase, Klotho-related protein, KLrP, lysosomal glucosylceramidase, microsomal bile acid beta-glucosidase, N-acylsphingosyl-1-O-beta-D-glucoside:glucohydrolase, neutral beta-glycosylceramidase, neutral glucosylceramidase, NLGase, non-lysosomal beta-glucosylceramidase, non-lysosomal glucosylceramidase, nonlysosomal beta-glucosidase, nonlysosomal glucosylceramidase, psychosine hydrolase, taliglucerase alfa, velaglucerase alfa, Vpriv

ECTree

     3 Hydrolases
         3.2 Glycosylases
             3.2.1 Glycosidases, i.e. enzymes that hydrolyse O- and S-glycosyl compounds
                3.2.1.45 glucosylceramidase

Activating Compound

Activating Compound on EC 3.2.1.45 - glucosylceramidase

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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
(3aR,4S,5R,6R,7S,7aS)-2-(3-phenylpropylimino)-octahydro-1H-benzo[d]imidazole-4,5,6,7-tetraol
-
Gcase activity increase: 1.9fold and 1.4fold in N370S and L44P, respectively, lymphoblasts from Gaucher patients
(3aR,4S,5R,6R,7S,7aS)-2-(nonylimino)-octahydro-1H-benzo[d]imidazole-4,5,6,7-tetraol
-
Gcase activity increase: 1.8fold and 1.4fold in N370S and L44P, respectively, lymphoblasts from Gaucher patients
(3aR,4S,5R,6S,7R,7aR)-2-(nonylimino)-octahydrobenzo[d]oxazole-4,5,6,7-tetraol
-
Gcase activity increase: 1.8fold and 1.4fold in N370S and L44P, respectively, lymphoblasts from Gaucher patients
(3aR,4S,5R,6S,7R,7aS)-2-(nonylimino)-octahydrobenzo[d]oxazole-4,5,6,7-tetraol
-
Gcase activity increase: 1.7fold and 1.3fold in N370S and L44P, respectively, lymphoblasts from Gaucher patients
(3aRS,4SR,5RS,6SR,7RS,7aRS)- 2-(nonylimino)-octahydrobenzo[d]oxazole-4,5,6,7-tetraol
-
Gcase activity increase: 1.7fold in N370S lymphoblasts from Gaucher patients
(3aS,4S,5R,6S,7R,7aR)-2-(nonylimino)-octahydrobenzo[d]oxazole-4,5,6,7-tetraol
-
Gcase activity increase: 1.4fold and 1.1fold in N370S and L44P, respectively, lymphoblasts from Gaucher patients
(3aS,4S,5R,6S,7R,7aS)-2-(nonylimino)-octahydrobenzo[d]oxazole-4,5,6,7-tetraol
-
Gcase activity increase: 2.1fold and 1.5fold in N370S and L44P, respectively, lymphoblasts from Gaucher patients
(3aSR,4SR,5RS,6SR,7RS,7aSR)-2-(nonylimino)-octahydrobenzo[d]oxazole-4,5,6,7-tetraol
-
Gcase activity increase: 1.7fold in N370S lymphoblasts from Gaucher patients
(R)-4-(3-phenoxypiperidin-1-yl)-2-(pyridin-3-yl)quinazoline
50% activation at 0.00365 mM
(R)-N-((2,3-dihydrobenzo[b][1,4]dioxin-2-yl)methyl)-N-methyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00237 mM
(S)-4-(3-phenoxypiperidin-1-yl)-2-(pyridin-3-yl)quinazoline
50% activation at 0.00475 mM
(S)-N-((2,3-dihydrobenzo[b][1,4]dioxin-2-yl)methyl)-N-methyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00149 mM
2-(2-(pyridin-3-yl)quinazolin-4-yl)-2,3,4,9-tetrahydro-1H-pyrido-[3,4-b]indole
50% activation at 0.00427 mM
2-(furan-2-yl)-N-methyl-N-(2-phenoxyethyl)quinazolin-4-amine
50% activation at 0.02173 mM
2-(furan-3-yl)-N-methyl-N-(2-phenoxyethyl)quinazolin-4-amine
50% activation at 0.00168 mM
4-(2-(pyridin-3-yl)quinazolin-4-yl)-1,4-oxazepane
50% activation at 0.04414 mM
4-(2-(pyridin-3-yl)quinazolin-4-yl)-2,3,4,5-tetrahydrobenzo[f ]-[1,4]oxazepine
50% activation at 0.00974 mM
4-(2-benzylpiperidin-1-yl)-2-(pyridin-3-yl)quinazoline
50% activation at 0.0047 mM
4-(3,4-dihydroisoquinolin-2(1H)-yl)-2-(pyridin-3-yl)quinazoline
50% activation at 0.00223 mM
4-(3-benzylpiperidin-1-yl)-2-(pyridin-3-yl)quinazoline
50% activation at 0.00518 mM
4-(3-phenoxypyrrolidin-1-yl)-2-(pyridin-3-yl)quinazoline
50% activation at 0.00117 mM
4-(3-phenylpiperidin-1-yl)-2-(pyridin-3-yl)quinazoline
50% activation at 0.00466 mM
acetazolamide
-
Activator protein
-
-
-
ambroxol
-
-
celastrol
-
significantly increases the quantity and catalytic activity of the enzyme by reducing protein degradation
dexamethasone
-
the pretreatment with dexamethasone has the effect of selectively increasing the uptake of wild-type and recombinant enzymes by the macrophages but not by the liver sinusoidal endothelial cells or hepatocyte cells in vitro, the dexamethasone pre-treatment increases the uptake of enzyme by kupffer cells and by splenic macrophages in vivo.
Dicetyl phosphate
-
-
glycoprotein
-
heat-stable
heat-stable factor
-
isofagomine
-
GlcCerase activity is enhanced in normal fibroblasts 1.3fold after treatment with 0.03 mM isofagomine for 5 days
Lubrol Px
-
activates when C12-4-nitrobenz-2-oxa-1,3-diazole labelled glucosylceramide is substrate at optimal concentration of 0.05-0.15%
monoclonal antibody
-
no. 122 mimicking the effect of saposin C
-
N-((1H-benzo[d]imidazol-2-yl)methyl)-N-methyl-2-(pyridin-3-yl)-quinazolin-4-amine
50% activation at 0.00792 mM
N-((2,3-dihydrobenzo[b][1,4]dioxin-6-yl)methyl)-N-methyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00488 mM
N-(2-(benzyloxy)ethyl)-N-methyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.01687 mM
N-(2-phenoxyethyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00156 mM
N-(2-phenoxyethyl)-N-propyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00318 mM
N-(4-ethynylphenyl)-5,7-dimethylpyrazolo[1,5-a]pyrimidine-3-carboxamide
NCGC00188758
N-(benzo[c][1,2,5]thiadiazol-5-ylmethyl)-N-methyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00184 mM
N-(benzo[d]oxazol-2-ylmethyl)-N-methyl-2-(pyridin-3-yl)-quinazolin-4-amine
50% activation at 0.01607 mM
N-(benzo[d]thiazol-2-ylmethyl)-N-methyl-2-(pyridin-3-yl)-quinazolin-4-amine
50% activation at 0.0069 mM
N-(benzo[d][1,3]dioxol-5-ylmethyl)-N-methyl-2-(pyridin-3-yl)-quinazolin-4-amine
50% activation at 0.00163 mM
N-(n-butyl)deoxynojirimycin
-
other names are miglustat and Zavesca, about 20% increase of wild type enzyme activity at 0.0025 and 0.005 mM
N-(n-nonyl) deoxynojirimycin
-
1.3-1.4fold increase in activity is observed for the wild type enzyme at 0.02 mM
N-cyclohexyl-N-methyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00236 mM
N-cyclopentyl-N-methyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00636 mM
N-ethyl-N-(2-phenoxyethyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00538 mM
N-methyl-2-(pyridin-3-yl)-N-(quinoxalin-2-ylmethyl)quinazolin-4-amine
50% activation at 0.00163 mM
N-methyl-N-((2-methylisoindolin-5-yl)methyl)-2-(pyridin-3-yl)-quinazolin-4-amine
50% activation at 0.01491 mM
N-methyl-N-(1-phenoxypropan-2-yl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00752 mM
N-methyl-N-(2-phenoxyethyl)-2-(pyridin-2-yl)quinazolin-4-amine
50% activation at above 0.05 mM
N-methyl-N-(2-phenoxyethyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00355 mM
N-methyl-N-(2-phenoxyethyl)-2-(pyridin-4-yl)quinazolin-4-amine
50% activation at 0.00289 mM
N-methyl-N-(2-phenoxyethyl)-2-(pyrimidin-5-yl)quinazolin-4-amine
50% activation at 0.00089 mM
N-methyl-N-(2-phenoxypropyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00897 mM
N-methyl-N-(3-phenoxypropyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00616 mM
N-methyl-N-(3-phenylpropyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00501 mM
N-methyl-N-(4-phenylbutyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00399 mM
N-methyl-N-(naphthalen-2-ylmethyl)-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.0038 mM
N-methyl-N-phenethyl-2-(pyridin-3-yl)quinazolin-4-amine
50% activation at 0.00139 mM
N-nonyl-deoxynojirimycin
-
Gcase activity increase: 1.9fold in N370S lymphoblasts from Gaucher patients
phosphatidylglycerol
phosphatidylinositol
-
acidic, unsaturated phospholipid interfaces interact with acid beta-glucosidase to conform the enzyme into an active structure
phosphatidylserine
Phospholipid
-
the full catalytic activity requires phospholipid interfaces
saposin C
-
saposin-A
-
saposin-C
-
sodium cholate
-
activates when C12-4-nitrobenz-2-oxa-1,3-diazole labelled glucosylceramide is substrate at concentration of optimal 0.5%
sodium taurocholate
sodium taurodeoxycholate
-
-
Triton X-100
Tween 20
-
activates when C12-4-nitrobenz-2-oxa-1,3-diazole labelled glucosylceramide is substrate at optimal concentration of 0.05-0.15%
additional information
-