Information on EC 3.2.1.52 - beta-N-acetylhexosaminidase and Organism(s) Homo sapiens

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The taxonomic range for the selected organisms is: Homo sapiens

The enzyme appears in selected viruses and cellular organisms

EC NUMBER
COMMENTARY hide
3.2.1.52
-
RECOMMENDED NAME
GeneOntology No.
beta-N-acetylhexosaminidase
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
O-glycosyl bond hydrolysis
-
-
-
-
PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
anhydromuropeptides recycling I
-
-
anhydromuropeptides recycling II
-
-
chitin degradation II (Vibrio)
-
-
chitin degradation III (Serratia)
-
-
Other glycan degradation
-
-
Various types of N-glycan biosynthesis
-
-
Amino sugar and nucleotide sugar metabolism
-
-
Glycosaminoglycan degradation
-
-
Glycosphingolipid biosynthesis - globo and isoglobo series
-
-
Glycosphingolipid biosynthesis - ganglio series
-
-
Metabolic pathways
-
-
SYSTEMATIC NAME
IUBMB Comments
beta-N-acetyl-D-hexosaminide N-acetylhexosaminohydrolase
Acts on N-acetylglucosides and N-acetylgalactosides.
CAS REGISTRY NUMBER
COMMENTARY hide
9012-33-3
c.f. EC 3.2.1.165
9027-52-5
deleted
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
-
Hex is linked to osteoarthritis and lysosomal storage disorders
physiological function
-
Hex is the predominant glycosidase released by chondrocytes to degrade glycosaminoglycan
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
3,3-dichlorophenylsulfonphthaleinyl-N-acetyl-beta-D-glucosaminide + H2O
?
show the reaction diagram
-
-
-
-
?
4-methylcoumar-7-yl-beta-N-acetyl-D-glucosamine + H2O
?
show the reaction diagram
-
-
-
-
?
4-methylcoumar-7-yl-beta-N-fluoroacetyl-D-glucosamine + H2O
?
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl beta-N-acetylglucosamine + H2O
?
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-galactopyranoside + H2O
4-methylumbelliferone + 2-acetamido-2-deoxy-beta-D-galactopyranoside
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside + H2O
4-methylumbelliferone + 2-acetamido-2-deoxy-beta-D-glucopyranoside
show the reaction diagram
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside + H2O
?
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside 6-sulfate + H2O
?
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside-6-sulfate + H2O
4-methylumbelliferone + 2-acetamido-2-deoxy-beta-D-glucopyranoside-6-sulfate
show the reaction diagram
4-methylumbelliferyl-6-sulfo-N-acetyl-beta-D-glucosaminide + H2O
?
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-beta-N-acetylglucosamine + H2O
4-methylumbelliferol + N-acetyl-D-glucosamine
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-beta-N-acetylglucosamine + H2O
4-methylumbelliferone + beta-N-acetylglucosamine
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-beta-N-acetylglucosamine-6-sulfate + H2O
4-methylumbelliferol + N-acetyl-D-glucosamine-6-sulfate
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl-N-acetyl-beta-D-glucosaminide + H2O
4-methylumbelliferone + N-acetyl-beta-D-glucosamine
show the reaction diagram
-
-
-
-
?
4-nitrophenyl N-acetyl-beta-D-glucosamine + H2O
4-nitrophenol + N-acetyl-beta-D-glucosamine
show the reaction diagram
-
-
-
-
?
4-nitrophenyl-2-acetamido-2-deoxy-beta-D-glucopyranoside + H2O
4-nitrophenol + N-acetyl-D-glucosamine
show the reaction diagram
-
-
-
-
?
4-nitrophenyl-beta-N-acetyl-D-galactosaminide + H2O
p-nitrophenol + N-acetyl-beta-D-galactosamine
show the reaction diagram
-
-
-
-
?
4-nitrophenyl-beta-N-acetyl-D-glucosaminide + H2O
4-nitrophenol + N-acetyl-beta-D-glucosamine
show the reaction diagram
-
-
-
-
?
4-nitrophenyl-beta-N-acetyl-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-beta-D-glucosamine
show the reaction diagram
-
-
-
-
?
globotetraosylceramide + H2O
globotriaosylceramide + GalNAc
show the reaction diagram
-
-
-
?
GM2 ganglioside + H2O
?
show the reaction diagram
-
-
-
-
?
GM2 ganglioside + H2O
GM3 ganglioside + N-acetyl-beta-D-galactosamine
show the reaction diagram
-
-
-
-
ir
GM2 ganglioside + H2O
N-acetyl-D-galactosamine
show the reaction diagram
-
-
-
?
N-acetyl-beta-D-hexosaminides + H2O
N-acetyl-D-hexosamines
show the reaction diagram
N-acetyl-galactosaminide-hexasaccharide + H2O
?
show the reaction diagram
-
-
-
-
?
p-nitrophenyl-beta-2-acetamido-2-deoxy-D-galactopyranoside + H2O
2-acetamido-2-deoxy-D-galactose + p-nitrophenol
show the reaction diagram
-
-
-
?
p-nitrophenyl-beta-2-acetamido-2-deoxy-D-glucopyranoside + H2O
2-acetamido-2-deoxy-D-glucose + p-nitrophenol
show the reaction diagram
p-nitrophenyl-GlcNAc + H2O
?
show the reaction diagram
-
-
-
-
?
additional information
?
-
NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
N-acetyl-beta-D-hexosaminides + H2O
N-acetyl-D-hexosamines
show the reaction diagram
additional information
?
-
INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
(1E)-2-nitro-1H-indene-1,3(2H)-dione oxime
-
-
(1Z)-2-nitro-1H-indene-1,3(2H)-dione oxime
-
-
(2E)-3-(3,4-dihydroisoquinolin-2(1H)-yl)-2-(2-thienylcarbonyl)acrylonitrile
-
-
(3E)-1-methyl-3-([[3-(trifluoromethyl)phenyl]amino]methylene)-1H-thieno[3,2-c][1,2]thiazin-4(3H)-one 2,2-dioxide
-
-
1,1'-(1,3-dioxo-2-phenylpropane-1,3-diyl)bis(2,3-dihydro-1H-indole)
-
-
1,2-dideoxy-2'-butyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
1,2-dideoxy-2'-ethyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
1,2-dideoxy-2'-isobutyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
1,2-dideoxy-2'-isopropyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
1,2-dideoxy-2'-methyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
an inhibitor that mimics the oxazoline intermediate proposed in the catalytic mechanism of family 20 glycoside hydrolases, is shown to act as a potent competitive inhibitor. About 17000fold more tightly than GlcNAc
1,2-dideoxy-2'-pentyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
1,2-dideoxy-2'-propyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
1-acetamido-2-deoxy-D-glucono-1,5-lactone
-
-
1-[2-(1H-benzimidazol-2-yl)phenyl]-3-[4-(benzyloxy)phenyl]urea
-
-
1-[5-acetyl-2-[4-(ethoxycarbonyl)piperidin-1-yl]phenyl]-1-methyldiazenium
-
-
2,2'-(iminodiethane-2,1-diyl)bis(1H-benzo[de]isoquinoline-1,3(2H)-dione)
-
-
2-(2-hydroxypropyl)-1H-benzo[de]isoquinoline-1,3(2H)-dione
-
-
2-(2-pyridin-2-ylethyl)-1H-benzo[de]isoquinoline-1,3(2H)-dione
-
-
2-(4-chlorobenzyl)-5-methyl-2,6-dihydro-1H-pyrrolo[3,4-d]pyridazin-1-one
-
-
2-(tetrahydrofuran-2-ylmethyl)-1H-benzo[de]isoquinoline-1,3(2H)-dione
-
-
2-acetamido-1,2,4-trideoxy-1,4-imino-L-arabinitol
-
potent noncompetitive inhibitor
2-acetamido-1,2,5-trideoxy-1,5-imino-D-glucitol
-
-
2-acetamido-1,2-dideoxy-1,5-imino-D-glucitol
-
competitive inhibitor
2-amino-6-(methoxymethyl)pyrimidin-4-ol
-
-
2-hydroxy-3-phenyl-4H-pyrimido[2,1-b][1,3]benzothiazol-4-one
-
-
3,5-bis(4-fluorophenyl)-1-phenyl-4,5-dihydro-1H-pyrazole
-
-
3-methoxy-1-(3,4,5-trimethoxyphenyl)-9,10-dihydrophenanthrene-2-carbonitrile
-
-
3-[2-(benzyloxy)-5-fluorophenyl]-5-(4-tert-butylphenyl)-1-(2,4-dichlorophenyl)-4,5-dihydro-1H-pyrazole
-
-
4,5-dihydronaphtho[1,2-b]thiophene-2-carbohydrazide
-
-
4-hydroxy-3-(methylthio)-N'-[(1E)-2-thienylmethylene]-4,5,6,7-tetrahydro-2-benzothiophene-1-carbohydrazide
-
-
4-[(E)-2-phenylvinyl]benzaldehyde O-(anilinocarbonyl)oxime
-
-
4-[4-(phenylethynyl)phenyl]-N-[4-(trifluoromethoxy)phenyl]butanamide
-
-
5-(3-nitrophenyl)-2-furaldehyde N-(3-fluorophenyl)thiosemicarbazone
-
-
5-(4-chloro-1-methyl-1H-pyrazol-3-yl)-4,5-dihydro-1,3,4-thiadiazole-2-thiol
-
-
acetate
Ag+
-
enzyme incubated before addition of substrate: with 0.0025 mM 18% of activity remains
ascorbate
-
only in presence of Cu2+
Cu2+
-
only in presence of ascorbate
elinafide
-
-
ethyl 3-(N-[[(4-chlorophenyl)amino]carbonyl]-S-methylsulfinimidoyl)-6,7-dihydro-2-benzothiophene-1-carboxylate
-
-
ethyl 5-[[3-(trifluoromethyl)phenyl]amino]pyrazolo[1,5-a]quinazoline-3-carboxylate
-
-
Fe2+
-
with 0.2 mM 28% of activity remains, citrate buffer, not inhibitory in succinate or acetate buffer
galactosamine
-
with 30 mM 84.3-89.3% of liver enzyme type A and 79.6-87.8% of liver enzyme type B remains
GalNAc-isofagomine
-
-
GlcNAc
-
-
glucosamine
-
with 30 mM 72.2-76.3% of liver enzyme type A and 65.7-73.3% of liver enzyme type B remains
N-((3S,4R,5S)-1-(7-(bis(4-methoxybenzyl)amino)heptyl)-4,5-dihydroxy-5-(hydroxymethyl)piperidin-3-yl)acetamide
-
-
N-((3S,4R,5S)-4,5-dihydroxy-5-(hydroxymethyl)-1-(7-(4-methoxybenzylamino)heptyl)piperidin-3-yl)acetamide
-
-
N-(3,5-dichlorophenyl)-2-[[4-(3,4-dimethylthieno[2,3-b]thien-2-yl)pyrimidin-2-yl]thio]acetamide
-
-
N-(7-((3S,4R,5S)-5-acetamido-3,4-dihydroxy-3-(hydroxymethyl)piperidin-1-yl)heptyl)-5-(2-oxohexahydro-1H-thieno[3,4-d]imidazol-4-yl)pentanamide
-
-
N-acetyl-D-galactosamine
N-acetyl-D-glucosamine
N-acetylglucosamine thiazoline
-
-
N-benzyl-2-acetamido-1,2,4-trideoxy-1,4-imino-D-arabinitol
-
weak competitive inhibitor
N-benzyl-2-acetamido-1,2,4-trideoxy-1,4-imino-L-arabinitol
-
potent noncompetitive inhibitor, inhibits human placenta beta-N-acetylhexosaminidase 2.5fold better than DNJ-NAc 13
N-[(1S,2R,5R,6R)-2-amino-5,6-dihydroxy-4-(hydroxymethyl)-cyclohex-3-en-1-yl]acetamide hydrochloride
-
1-acetamido derivative of 6-epi-valienamine, route enabling synthesis described
N-[1(7-amino-heptyl)]-2-acetamido-1,2,5-trideoxy-1,5-imino-D-glucitol
-
competitive inhibitor
N-[1(7-azido-heptyl)]-2-acetamido-1,2,5-trideoxy-1,5-imino-D-glucitol
-
-
naphthalene-1,4-diyl bis(trifluoroacetate)
-
-
O-(2-acetamido-2-deoxy-D-glucopyranosylidene) amino-N-phenylcarbamate
-
PUGNAc
O-(2-acetamido-2-deoxy-D-glucopyranosylidene)amino N-phenylcarbamate
-
inhibitor of both human O-GlcNAcase and human beta-hexosaminidase
O-(2-deoxy-2-butamido-D-glucopyranosylidene)amino N-phenylcarbamate
-
inhibitor of both human O-GlcNAcase and human beta-hexosaminidase
O-(2-deoxy-2-hexamido-D-glucopyranosylidene)amino N-phenylcarbamate
-
inhibitor of both human O-GlcNAcase and human beta-hexosaminidase
O-(2-deoxy-2-isobutamido-D-glucopyranosylidene)amino N-phenylcarbamate
-
inhibitor of both human O-GlcNAcase and human beta-hexosaminidase
O-(2-deoxy-2-isovaleramido-D-glucopyranosylidene)amino N-phenylcarbamate
-
inhibitor of both human O-GlcNAcase and human beta-hexosaminidase
O-(2-deoxy-2-propamido-D-glucopyranosylidene)amino N-phenylcarbamate
-
inhibitor of both human O-GlcNAcase and human beta-hexosaminidase
O-(2-deoxy-2-valeramido-D-glucopyranosylidene)amino N-phenylcarbamate
-
inhibitor of both human O-GlcNAcase and human beta-hexosaminidase
p-chloromercuribenzoate
-
form A: 93% inhibition with 0.1 mM, form P: 98% inhibition with 0.1 mM
p-Chloromercuriphenylsulfonate
-
with 1 mM 38.3% of liver enzyme type S, 14.2% of brain enzyme type S, 1.9-2.2% of liver enzyme type A and 2.6-9.9% of liver enzyme type B remains
pyrimethamine
-
-
S-(thiophen-3-ylmethyl) (2,2'-bithiophen-5-ylcarbonyl)thiocarbamate
-
-
thioguanine
-
-
additional information
-
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
bovine serum albumin
-
-
-
cysteine
-
prevents inhibition by Ag2+-ions; prevents inhibition by Hg2+-ions
D-galactosamine
-
with 30 mM the activity of liver and brain enzymes reaches 135% and 114.3%
D-glucosamine
-
with 30 mM the activity of liver and brain enzymes reaches 135% and 110.7%
GM12 activator protein
-
-
-
Human serum albumin
-
-
-
N-benzyl-2-acetamido-1,2,4-trideoxy-1,4-imino-L-arabinitol
-
enhancement of beta-N-acetylhexosaminidase B activity at higher concentrations of inhibitor, whereas beta-N-acetylhexosaminidase A and/or beta-N-acetylhexosaminidase S activity are increased at much lower concentrations
taurocholate
-
concentrations of 0.05 M and 0.1 M cause 140% and 160% of activity
additional information
-
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.88
4-methylcoumar-7-yl-beta-N-acetyl-D-glucosamine
-
-
3.8
4-methylcoumar-7-yl-beta-N-fluoroacetyl-D-glucosamine
-
-
0.108
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-galactopyranoside
-
-
0.55 - 1
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside
1.8
4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside-6-sulfate
-
-
-
0.3 - 0.4
4-methylumbelliferyl-beta-N-acetylgalactosaminide
-
liver and brain enzymes type S
0.8 - 0.9
4-methylumbelliferyl-beta-N-acetylglucosaminide
-
liver and brain enzymes type S
-
0.046 - 0.12
complex of GM2 activator protein and GM2 ganglioside
-
0.17
p-nitrophenyl-beta-2-acetamido-2-deoxy-D-galactopyranoside
-
-
0.83
p-nitrophenyl-beta-2-acetamido-2-deoxy-D-glucopyranoside
-
-
Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.0061
(1E)-2-nitro-1H-indene-1,3(2H)-dione oxime
-
-
4.6
1,2-dideoxy-2'-butyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
0.032
1,2-dideoxy-2'-ethyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
4
1,2-dideoxy-2'-isobutyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
0.72
1,2-dideoxy-2'-isopropyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
0.00007
1,2-dideoxy-2'-methyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
11
1,2-dideoxy-2'-pentyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
0.34
1,2-dideoxy-2'-propyl-alpha-D-glucopyranoso-[2,1-d]-DELTA2'-thiazoline
-
-
0.0008
2,2'-(iminodiethane-2,1-diyl)bis(1H-benzo[de]isoquinoline-1,3(2H)-dione)
-
-
0.015
2-acetamido-1,2,4-trideoxy-1,4-imino-L-arabinitol
-
-
0.00054
2-acetamido-1,2,5-trideoxy-1,5-imino-D-glucitol
-
pH 4.25, 30°C, isozyme Hex B
0.0095
2-acetamido-1,2-dideoxy-1,5-imino-D-glucitol
-
-
1.2
GlcNAc
-
-
0.00000069
N-((3S,4R,5S)-1-(7-(bis(4-methoxybenzyl)amino)heptyl)-4,5-dihydroxy-5-(hydroxymethyl)piperidin-3-yl)acetamide
-
pH 4.25, 30°C, isozyme Hex B
0.0000012
N-((3S,4R,5S)-4,5-dihydroxy-5-(hydroxymethyl)-1-(7-(4-methoxybenzylamino)heptyl)piperidin-3-yl)acetamide
-
pH 4.25, 30°C, isozyme Hex B
0.0000267
N-(7-((3S,4R,5S)-5-acetamido-3,4-dihydroxy-3-(hydroxymethyl)piperidin-1-yl)heptyl)-5-(2-oxohexahydro-1H-thieno[3,4-d]imidazol-4-yl)pentanamide
-
pH 4.25, 30°C, isozyme Hex B
0.0061
N-acetylglucosamine thiazoline
-
-
0.18
N-benzyl-2-acetamido-1,2,4-trideoxy-1,4-imino-D-arabinitol
-
-
0.0037
N-benzyl-2-acetamido-1,2,4-trideoxy-1,4-imino-L-arabinitol
-
-
0.0062
N-[(1S,2R,5R,6R)-2-amino-5,6-dihydroxy-4-(hydroxymethyl)-cyclohex-3-en-1-yl]acetamide hydrochloride
-
substrate concentration 0.5 mM, two enzyme concentrations of 0.0406 and 0.012 micrograms per microliter
0.0000021
N-[1(7-amino-heptyl)]-2-acetamido-1,2,5-trideoxy-1,5-imino-D-glucitol
-
pH 4.25, 30°C, isozyme Hex B
0.0017
N-[1(7-azido-heptyl)]-2-acetamido-1,2,5-trideoxy-1,5-imino-D-glucitol
-
pH 4.25, 30°C, isozyme Hex B
0.000036
O-(2-acetamido-2-deoxy-D-glucopyranosylidene) amino-N-phenylcarbamate
-
-
0.000036
O-(2-acetamido-2-deoxy-D-glucopyranosylidene)amino N-phenylcarbamate
-
-
0.026
O-(2-deoxy-2-butamido-D-glucopyranosylidene)amino N-phenylcarbamate
-
-
0.02
O-(2-deoxy-2-isobutamido-D-glucopyranosylidene)amino N-phenylcarbamate
-
-
0.0012
O-(2-deoxy-2-propamido-D-glucopyranosylidene)amino N-phenylcarbamate
-
33fold increase in the KI value for beta-hexosaminidase as compared to the parent O-(2-acetamido-2-deoxy-D-glucopyranosylidene)amino N-phenylcarbamate
0.22
O-(2-deoxy-2-valeramido-D-glucopyranosylidene)amino N-phenylcarbamate
-
-
0.013
pyrimethamine
-
-
additional information
O-(2-deoxy-2-hexamido-D-glucopyranosylidene)amino N-phenylcarbamate
IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
27
(1E)-2-nitro-1H-indene-1,3(2H)-dione oxime
Homo sapiens;
-
-
0.0018
(1Z)-2-nitro-1H-indene-1,3(2H)-dione oxime
Homo sapiens;
-
-
0.042
(2E)-3-(3,4-dihydroisoquinolin-2(1H)-yl)-2-(2-thienylcarbonyl)acrylonitrile
Homo sapiens;
-
-
0.27
(3E)-1-methyl-3-([[3-(trifluoromethyl)phenyl]amino]methylene)-1H-thieno[3,2-c][1,2]thiazin-4(3H)-one 2,2-dioxide
Homo sapiens;
-
-
0.034
1,1'-(1,3-dioxo-2-phenylpropane-1,3-diyl)bis(2,3-dihydro-1H-indole)
Homo sapiens;
-
-
0.012
1-[2-(1H-benzimidazol-2-yl)phenyl]-3-[4-(benzyloxy)phenyl]urea
Homo sapiens;
-
-
0.065
1-[5-acetyl-2-[4-(ethoxycarbonyl)piperidin-1-yl]phenyl]-1-methyldiazenium
Homo sapiens;
-
-
0.0002 - 6
2,2'-(iminodiethane-2,1-diyl)bis(1H-benzo[de]isoquinoline-1,3(2H)-dione)
0.031
2-(2-hydroxypropyl)-1H-benzo[de]isoquinoline-1,3(2H)-dione
Homo sapiens;
-
-
0.108
2-(2-pyridin-2-ylethyl)-1H-benzo[de]isoquinoline-1,3(2H)-dione
Homo sapiens;
-
-
0.0058 - 29
2-(4-chlorobenzyl)-5-methyl-2,6-dihydro-1H-pyrrolo[3,4-d]pyridazin-1-one
0.138
2-(tetrahydrofuran-2-ylmethyl)-1H-benzo[de]isoquinoline-1,3(2H)-dione
Homo sapiens;
-
-
0.025 - 0.044
2-amino-6-(methoxymethyl)pyrimidin-4-ol
0.072
2-hydroxy-3-phenyl-4H-pyrimido[2,1-b][1,3]benzothiazol-4-one
Homo sapiens;
-
-
0.0065
3,5-bis(4-fluorophenyl)-1-phenyl-4,5-dihydro-1H-pyrazole
Homo sapiens;
-
-
0.035
3-methoxy-1-(3,4,5-trimethoxyphenyl)-9,10-dihydrophenanthrene-2-carbonitrile
Homo sapiens;
-
-
0.162
3-[2-(benzyloxy)-5-fluorophenyl]-5-(4-tert-butylphenyl)-1-(2,4-dichlorophenyl)-4,5-dihydro-1H-pyrazole
Homo sapiens;
-
-
0.024
4,5-dihydronaphtho[1,2-b]thiophene-2-carbohydrazide
Homo sapiens;
-
-
0.03
4-hydroxy-3-(methylthio)-N'-[(1E)-2-thienylmethylene]-4,5,6,7-tetrahydro-2-benzothiophene-1-carbohydrazide
Homo sapiens;
-
-
0.18
4-[(E)-2-phenylvinyl]benzaldehyde O-(anilinocarbonyl)oxime
Homo sapiens;
-
-
0.071
4-[4-(phenylethynyl)phenyl]-N-[4-(trifluoromethoxy)phenyl]butanamide
Homo sapiens;
-
-
0.042
5-(3-nitrophenyl)-2-furaldehyde N-(3-fluorophenyl)thiosemicarbazone
Homo sapiens;
-
-
0.079
5-(4-chloro-1-methyl-1H-pyrazol-3-yl)-4,5-dihydro-1,3,4-thiadiazole-2-thiol
Homo sapiens;
-
-
0.042
elinafide
Homo sapiens;
-
-
0.039
ethyl 3-(N-[[(4-chlorophenyl)amino]carbonyl]-S-methylsulfinimidoyl)-6,7-dihydro-2-benzothiophene-1-carboxylate
Homo sapiens;
-
-
0.045
ethyl 5-[[3-(trifluoromethyl)phenyl]amino]pyrazolo[1,5-a]quinazoline-3-carboxylate
Homo sapiens;
-
-
0.02
N-(3,5-dichlorophenyl)-2-[[4-(3,4-dimethylthieno[2,3-b]thien-2-yl)pyrimidin-2-yl]thio]acetamide
Homo sapiens;
-
-
0.093
naphthalene-1,4-diyl bis(trifluoroacetate)
Homo sapiens;
-
-
0.008
pyrimethamine
Homo sapiens;
-
-
0.029
S-(thiophen-3-ylmethyl) (2,2'-bithiophen-5-ylcarbonyl)thiocarbamate
Homo sapiens;
-
-
0.13
thioguanine
Homo sapiens;
-
-
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
0.0012 - 0.0025
-
substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside-6-sulfate
0.0024 - 0.01
-
light lysosomal fraction, I-cell
0.0032 - 0.008
-
light lysosomal fraction, normal
0.0033 - 0.0066
-
light lysosomal fraction, Pseudo-Hurler
0.0051 - 0.0079
-
heavy lysosomal fraction, I-cell
0.0061 - 0.0075
-
heavy lysosomal fraction, Pseudo-Hurler
0.0061 - 0.0211
-
heavy lysosomal fraction, normal
0.123
-
substrate: 4-methylumbelliferyl-beta-N-acetylglucosaminide, brain enzyme type S
0.16
-
recombinant enzyme, substrate: 4-methylumbelliferyl-6-sulfo-N-acetyl-beta-D-glucosaminide
0.28
-
recombinant enzyme, substrate: 4-methylumbelliferyl-N-acetyl-beta-D-glucosaminide
0.447
-
substrate: 4-methylumbelliferyl-beta-N-acetylglucosaminide, liver enzyme type S
77.84
-
substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside, normal enzyme
90.63
-
substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside, I-cell enzyme
130
-
hexosaminidase I2, substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside
150
-
hexosaminidase A, substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside
320
-
hexosaminidase B, substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside
additional information
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
3.5 - 4
-
substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside-6-sulfate
4.25
-
assay at
4.5 - 5
-
-
4.7
-
in a citrate-phosphate buffer
5
-
substrate: 4-methylumbelliferyl-beta-N-acetylglucosaminide or -acetylgalactosaminide, liver and brain enzymes type S
additional information
-
-
pH RANGE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
3.5 - 5.1
-
pH 3.5: about 45% of maximal activity, pH 5.1: about 65% of maximal activity
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
30
-
assay at
37
-
assay at
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
-
elevated activity of N-acetyl-beta-hexosaminidase in subjects with asthma
Manually annotated by BRENDA team
-
about 2fold increase in N-acetyl-beta-D-hexosaminidase activity compared to normal adult retroauricular skin
Manually annotated by BRENDA team
-
fetal membrane
Manually annotated by BRENDA team
-
a similar pattern in activity of isoforms of isoenzymes A and B in normal and cancerous renal tissue is observed
Manually annotated by BRENDA team
-
-
Manually annotated by BRENDA team
-
desalinated
Manually annotated by BRENDA team
-
total activity of N-acetyl-beta-hexosaminidase (HEX), as well as HEX A and HEX B, in pleomorphic adenoma is significantly higher compared with normal human salivary gland
Manually annotated by BRENDA team
-
a similar pattern in activity of isoforms of isoenzymes A and B in normal and cancerous renal tissue is observed
Manually annotated by BRENDA team
-
no significant difference is found in total enzyme activity between normozoospermic controls and patients with secretory azoospermia. Ioenzyme A activity is significantly lower and the ratio between total enzyme activity and isoenzyme A activity is significantly higher in azoospermic patients compared to controls. This difference does not represent a useful marker of secretory azoospermia
Manually annotated by BRENDA team
-
normal adult retroauricular skin
Manually annotated by BRENDA team
additional information
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
PDB
SCOP
CATH
UNIPROT
ORGANISM
Homo sapiens;
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
26000
-
1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE
29500
-
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme
54000
-
1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE
55600
-
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme
56000
-
hexosaminidase A, major components: 1 * 56000 + 1 * 29000, minor components: 26000 - 22000, SDS-PAGE
58000
-
1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme
59600
-
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme
66000
-
major Hex I2 form: alpha, beta, 1 * 66000 + 1 * 66000, beta chain 2 residues short of being identical to alpha chain, SDS-PAGE
68000
-
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme
100000
-
hexosaminidase A
100000 - 110000
-
hexosaminidase I2, gradient gel electrophoresis
105000
-
sedimentation equilibrium using untreated enzyme, carboxymethylated enzyme in 6 M guanidine-HCl, meniscus depletion experiments with fresh enzyme prepatation at pH 11.5
107000
-
gel filtration
112000
-
-
120000
-
enzyme after dialysis and freeze-drying, 6 M guanidine-HCl, pH 7.2
130000
-
Hex I2, gel filtration
140000
-
gel filtration
152000
-
brain enzyme type S, gel filtration
154000
-
liver enzyme type S, gel filtration
260000
-
pH 2.5, fresh enzyme prepatation, meniscus depletion experiments
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
heterodimer
-
-
tetramer
-
1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme
trimer
-
1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme
additional information
-
-
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
glycoprotein
Crystallization/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
hanging-drop method, crystal structure of beta-hexosaminidase B, alone at 2.4 A and in complex with the mechanistic inhibitor GalNAc-isofagomine at 2.2 A or N-acetyl-beta-D-glucosamine-thiazoline at 2.5 A resolution
-
hanging-drop vapour-diffusion method, crystal structure of a complex of beta-hexosaminidase B with a transition state analogue inhibitor 1-acetamido-2-deoxy-D-glucono-1,5-lactone at 2.3 A resolution
-
hexosaminidase B, space group P6122 i.e. enantiomorph, 3.2 A resolution
-
vapor diffusion method
-
TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
0 - 37
-
stable up to 24 h
50 - 52
-
Hex A is labile, Hex B is stable, at lower sample concentrations Hex B is less stable at 52°C
GENERAL STABILITY
ORGANISM
UNIPROT
LITERATURE
resistant to mechanical inactivation by homogenization or freezing and thawing
-
ORGANIC SOLVENT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
dimethyl sulfoxide
-
with 7.5% 14% of activity remains
dimethylformamide
-
with 7.5% 9% of activity remains
dithiothreitol
-
reduction, produces fragments of 63000, 56000, 36000 and 28000 Da
STORAGE STABILITY
ORGANISM
UNIPROT
LITERATURE
-70°C, hexosaminidase A, B and I2 separated from each other
-
4°C, liver enzyme type S, concentrated by ultrafiltration to 0.18 mg/ml, in 0.04 citrate buffer and in presence of 0.2 mg/ml bovine serum albumin stable for at least 3 months
-
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
1500fold
-
195fold to homogeneity
-
hexosaminidase A, B and I2 purified 2699, 10320 and 13077fold
-
liver enzyme type S 13500fold, brain enzyme type S 5240fold, liver enzymes types A and B
-
partial
-
recombinant enzyme. HexA is purified with immobilized metal affinity column for the His tag attached to the beta-subunit
-
to homogeneity, normal enzyme 64863fold, I-cell enzyme 20140fold
-
Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
expressed in Escherichia coli
-
expressed in Escherichia coli BL21(DE3)pLysS cells
-
recombinant His-tagged HexA is produced in the methylotrophic yeast Ogataea minuta (alpha-1,6-mannosyltransferase-deficient). Genes encoding the alpha- and beta-subunits of HexA are integrated into the yeast cell, and the heterodimer is expressed together with its isozymes HexS (alphaalpha) and HexB (betabeta). A total of 57 mg of beta-hexosaminidase isozymes, of which 13 mg was HexA (alphabeta), is produced per liter of medium. The C terminus of the beta-subunit appears to be truncated in Ogataea minuta recombinant HexA
-
ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
betaAsp452Asn/betaLeu453Arg
-
the mutant enzyme of HexB exhibits more than 30fold increase in its ability to hydrolyze a 6-sulfated substrate and is able to hydrolyze GM2 ganglioside when the GM2 activator protein is replaced by sodium taurocholate
R178H
-
from patient with the genetic disease GM2-gangliosidosis B1 variant, mutated Hex A, altered active site of alpha subunit
R424Q
-
the mutant form of HexA shows about 3fold increase in its Km-value for the complex of GM2 activator protein and GM2 ganglioside
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
diagnostics
-
a spectrophotometric method for the determination of lysosomally derived N-acetyl-beta-D-hexosaminidase in synovial fluid on a microplate reader is optimized to improve its utility. The assay is sufficiently sensitive for small volumes of synovial fluid, and is useful for the clinical diagnosis of joint diseases
drug development
-
the enzyme is a target for GlcNAc-inspired iminocyclitiols drug development
medicine