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(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
2,4-dinitrophenyl-alpha-N-acetyl-D-glucosaminide + H2O
2,4-dinitrophenol + N-acetyl-D-glucosamine
25°C
-
-
?
2-nitrophenyl alpha-N-acetylglucosaminide + H2O
2-nitrophenol + alpha-N-acetylglucosamine
-
-
-
-
?
4-methylumbelliferyl 2-acetamido-2-deoxy-alpha-D-glucopyranoside + H2O
?
-
-
-
?
4-methylumbelliferyl 4-O-N-acetyl-alpha-D-glucosaminyl-beta-D-galactoside + H2O
N-acetyl-alpha-D-glucosamine + 4-methylumbelliferyl beta-D-galactosaminide
-
-
-
-
?
4-methylumbelliferyl 6-O-N-acetyl-alpha-D-glucosaminyl-beta-D-galactoside + H2O
N-acetyl-alpha-D-glucosamine + 4-methylumbelliferyl beta-D-galactosaminide
-
slow reaction
-
-
?
4-methylumbelliferyl N-acetyl-alpha-D-glucosaminide + H2O
4-methylumbelliferone + N-acetyl-alpha-D-glucosamine
4-methylumbelliferyl-2-acetamide-2-deoxy-alpha-D-glucopyranoside + H2O
4-methylumbelliferol + 2-acetamide-2-deoxy-alpha-D-glucopyranose
-
-
-
-
?
4-nitrophenyl N-acetyl-alpha-D-glucosaminide + H2O
4-nitrophenol + N-acetyl-alpha-D-glucosamine
-
slow reaction
-
-
?
4-nitrophenyl-alpha-N-acetyl-D-glucosaminide + H2O
4-nitrophenol + N-acetyl-alpha-D-glucose
25°C
-
-
?
4-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
N-acetyl-alpha-D-glucosamine + 4-nitrophenol
cell surface mucin + H2O
N-acetyl-alpha-D-glucosamine + ?
chitobiose + H2O
?
-
-
-
?
chitotetraose + H2O
?
-
-
-
?
chitotriose + H2O
?
-
-
-
-
?
heparan sulfate + H2O
N-acetyl-alpha-D-glucosamine
heparan sulfate + H2O
N-acetyl-alpha-D-glucosamine + ?
-
-
-
-
?
heparin + H2O
N-acetyl-alpha-D-glucosamine + ?
-
-
-
?
methylumbelliferyl-N-acetyl-alpha-D-glucosaminide + H2O
methylumbelliferone + N-acetyl-alpha-D-glucosamine
methylumbelliferyl-N-acetylglucosamine + H2O
methylumbelliferone + N-acetylglucosamine
-
-
-
?
methylumbelliferyl-N-acetylglucosamine-6-sulfate + H2O
methylumbelliferone + N-acetylglucosamine-6-sulfate
-
-
-
?
N-acetyl-alpha-D-Gluc-1,4-(1-methoxyphenyl)Gal + H2O
N-acetyl-alpha-D-glucosamine + 1-methoxyphenyl-Gal
O-(alpha-acetamido-2-deoxy-D-glucopyranosyl)-(1-3)-[L-6,3H]idoronic acid + H2O
?
-
-
-
?
o-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
o-nitrophenol + N-acetyl-alpha-D-glucosamine
p-nitrophenyl-1-thio-N-acetylglucosamine + H2O
?
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
porcine gastric mucin + H2O
N-acetyl-alpha-D-glucosamine + ?
UDP-N-acetyl-alpha-D-glucosamine + H2O
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
uridine-5'-diphospho-N-acetyl-alpha-D-glucosamine
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
additional information
?
-
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
4-methylumbelliferyl N-acetyl-alpha-D-glucosaminide + H2O
4-methylumbelliferone + N-acetyl-alpha-D-glucosamine
-
efficient substrate
-
-
?
4-methylumbelliferyl N-acetyl-alpha-D-glucosaminide + H2O
4-methylumbelliferone + N-acetyl-alpha-D-glucosamine
-
-
-
?
4-methylumbelliferyl N-acetyl-alpha-D-glucosaminide + H2O
4-methylumbelliferone + N-acetyl-alpha-D-glucosamine
-
-
-
-
?
4-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
N-acetyl-alpha-D-glucosamine + 4-nitrophenol
-
-
-
?
4-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
N-acetyl-alpha-D-glucosamine + 4-nitrophenol
-
-
-
?
cell surface mucin + H2O
N-acetyl-alpha-D-glucosamine + ?
-
-
-
?
cell surface mucin + H2O
N-acetyl-alpha-D-glucosamine + ?
-
-
-
?
heparan sulfate + H2O
N-acetyl-alpha-D-glucosamine
-
-
-
?
heparan sulfate + H2O
N-acetyl-alpha-D-glucosamine
-
-
-
?
methylumbelliferyl-N-acetyl-alpha-D-glucosaminide + H2O
methylumbelliferone + N-acetyl-alpha-D-glucosamine
-
-
-
?
methylumbelliferyl-N-acetyl-alpha-D-glucosaminide + H2O
methylumbelliferone + N-acetyl-alpha-D-glucosamine
-
-
-
?
N-acetyl-alpha-D-Gluc-1,4-(1-methoxyphenyl)Gal + H2O
N-acetyl-alpha-D-glucosamine + 1-methoxyphenyl-Gal
-
-
-
?
N-acetyl-alpha-D-Gluc-1,4-(1-methoxyphenyl)Gal + H2O
N-acetyl-alpha-D-glucosamine + 1-methoxyphenyl-Gal
-
-
-
?
o-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
o-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
o-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
o-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
o-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
o-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
p-nitrophenyl-N-acetyl-alpha-D-glucosaminide + H2O
p-nitrophenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
-
-
-
-
?
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
phenyl-N-acetyl-alpha-D-glucosaminide + H2O
phenol + N-acetyl-alpha-D-glucosamine
-
-
-
?
porcine gastric mucin + H2O
N-acetyl-alpha-D-glucosamine + ?
-
the enzyme specifically hydrolyzes alpha-linked N-acetylglucosamine at nonreducing terminus of O-glycan on gastric mucin
-
-
?
porcine gastric mucin + H2O
N-acetyl-alpha-D-glucosamine + ?
-
-
-
?
porcine gastric mucin + H2O
N-acetyl-alpha-D-glucosamine + ?
-
-
-
?
UDP-N-acetyl-alpha-D-glucosamine + H2O
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
-
-
-
?
UDP-N-acetyl-alpha-D-glucosamine + H2O
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
-
-
-
-
?
uridine-5'-diphospho-N-acetyl-alpha-D-glucosamine
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
-
-
-
?
uridine-5'-diphospho-N-acetyl-alpha-D-glucosamine
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
-
-
-
?
uridine-5'-diphospho-N-acetyl-alpha-D-glucosamine
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
-
-
-
?
uridine-5'-diphospho-N-acetyl-alpha-D-glucosamine
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
-
-
-
?
additional information
?
-
-
no activity with 4-nitrophenyl N-acetyl-beta-D-glucosaminide, 4-nitrophenyl N-acetyl-alpha-D-galactosaminide, 4-nitrophenyl N-acetyl-beta-D-galactosaminide, 4-nitrophenyl alpha-D-glucosaminide, 4-nitrophenyl beta-D-glucosaminide, 4-nitrophenyl alpha-D-galactosaminide, 4-nitrophenyl beta-D-galactosaminide, 4-nitrophenyl alpha-D-fucosaminide, 4-nitrophenyl beta-D-fucosaminide, 4-nitrophenyl alpha-D-mannosaminide, 4-nitrophenyl beta-D-mannosaminide, 4-nitrophenyl alpha-D-xylosaminide, and 4-nitrophenyl beta-D-xylosaminide. Alpha1,2- and alpha1,3-linked N-acetyl-alpha-D-glucosaminides are resistant to this enzyme
-
-
?
additional information
?
-
-
swollen chitin and oligosaccharide above (GlcNAc)4 are not hydrolyzed by the enzyme
-
?
additional information
?
-
no activity against any of the GlcNAc1,2-(1-methoxyphenyl)Gal, GlcNAc-1,3-(1-methoxyphenyl)Gal, GlcNAc-1,6-(1-methoxyphenyl)Gal, andGlcNAc-1,4-(1-methoxyphenyl)Glc substrates
-
-
?
additional information
?
-
-
no activity against any of the GlcNAc1,2-(1-methoxyphenyl)Gal, GlcNAc-1,3-(1-methoxyphenyl)Gal, GlcNAc-1,6-(1-methoxyphenyl)Gal, andGlcNAc-1,4-(1-methoxyphenyl)Glc substrates
-
-
?
additional information
?
-
no activity against any of the GlcNAc1,2-(1-methoxyphenyl)Gal, GlcNAc-1,3-(1-methoxyphenyl)Gal, GlcNAc-1,6-(1-methoxyphenyl)Gal, andGlcNAc-1,4-(1-methoxyphenyl)Glc substrates
-
-
?
additional information
?
-
-
mucopolysaccharidosis type IIIB is a lysosomal storage disorder characterized by the defective degradation of heparan sulfate due to a deficiency of alpha-N-acetylglucosaminidase. The clinical severity of mucopolysacchariddosis type IIIB ranges from an attenuated to severely affected Sanfilippo phenotype. Relationship between genotype, cellular biochemistry and clinical phenotype in a F48L/R297X compound heterozygous mucopolysaccharidosis type IIIB patient with an attenuated Sanfilippo phenotype
-
?
additional information
?
-
-
enzyme deficiency causes the Sanfilippo type B syndrome, i.e. the mucopolysaccharidosis type IIIB, a lysosomal storage disorder, phenotype, overview
-
-
?
additional information
?
-
-
enzyme deficiency causes the Sanfilippo type B syndrome, i.e. the mucopolysaccharidosis type IIIB, an autosomal recessive lysosomal storage disorder, overview
-
-
?
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(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
UDP-N-acetyl-alpha-D-glucosamine + H2O
uridine-5'-diphosphate + N-acetyl-alpha-D-glucosamine
-
-
-
?
additional information
?
-
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
involved in the degradation of heparin and heparan sulfate
-
-
?
(GlcNAcalpha(1-4))n + H2O
(GlcNAcalpha(1-4))n-1 + 2-(acetylamino)-2-deoxy-beta-D-glucopyranose
-
-
-
?
additional information
?
-
-
mucopolysaccharidosis type IIIB is a lysosomal storage disorder characterized by the defective degradation of heparan sulfate due to a deficiency of alpha-N-acetylglucosaminidase. The clinical severity of mucopolysacchariddosis type IIIB ranges from an attenuated to severely affected Sanfilippo phenotype. Relationship between genotype, cellular biochemistry and clinical phenotype in a F48L/R297X compound heterozygous mucopolysaccharidosis type IIIB patient with an attenuated Sanfilippo phenotype
-
?
additional information
?
-
-
enzyme deficiency causes the Sanfilippo type B syndrome, i.e. the mucopolysaccharidosis type IIIB, a lysosomal storage disorder, phenotype, overview
-
-
?
additional information
?
-
-
enzyme deficiency causes the Sanfilippo type B syndrome, i.e. the mucopolysaccharidosis type IIIB, an autosomal recessive lysosomal storage disorder, overview
-
-
?
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
0.74
2,4-dinitrophenyl-alpha-N-acetyl-D-glucosaminide
pH 7.3
0.043 - 0.61
2-nitrophenyl alpha-N-acetylglucosaminide
-
form I 0.067, form II 0.043
-
1.07 - 5.34
4-methylumbelliferyl 2-acetamido-2-deoxy-alpha-D-glucopyranoside
1.1
4-nitrophenyl-alpha-N-acetyl-D-glucosaminide
pH 7.3
4.3 - 7.8
4-nitrophenyl-N-acetyl-alpha-D-glucosaminide
0.13 - 0.22
methylumbelliferyl-N-acetyl-alpha-D-glucosaminide
0.001
methylumbelliferyl-N-acetylglucosamine
-
-
0.0035
methylumbelliferyl-N-acetylglucosamine-6-sulfate
-
-
0.2 - 0.28
N-acetyl-alpha-D-Gluc-1,4-(1-methoxyphenyl)Gal
0.0166
O-(alpha-acetamido-2-deoxy-D-glucopyranosyl)-(1-3)-[L-6,3H]idoronic acid
-
pH 4.5, 37°C, with bovine serum albumin
0.043 - 0.61
o-nitrophenyl-alpha-N-acetylglucosaminide
0.00055
p-nitrophenyl-1-thio-N-acetylglucosamine
-
-
0.13 - 0.3
p-nitrophenyl-alpha-N-acetylglucosaminide
0.12 - 1.6
phenyl-alpha-N-acetylglucosaminide
0.39
UDP-N-acetylglucosamine
-
0.58 in the presence of 0.025% bovine serum albumin
1.07
4-methylumbelliferyl 2-acetamido-2-deoxy-alpha-D-glucopyranoside
-
pH 4.5, 37°C, without bovine serum albumin
5.34
4-methylumbelliferyl 2-acetamido-2-deoxy-alpha-D-glucopyranoside
-
pH 4.5, 37°C, with bovine serum albumin
4.3
4-nitrophenyl-N-acetyl-alpha-D-glucosaminide
wild-type, pH 7.4, 37°C
7.8
4-nitrophenyl-N-acetyl-alpha-D-glucosaminide
C-terminal deletion mutant, pH 7.4, 37°C
0.13 - 0.2
methylumbelliferyl-N-acetyl-alpha-D-glucosaminide
-
-
0.22
methylumbelliferyl-N-acetyl-alpha-D-glucosaminide
-
purified enzyme
0.2
N-acetyl-alpha-D-Gluc-1,4-(1-methoxyphenyl)Gal
wild-type, pH 7.4, 37°C
0.28
N-acetyl-alpha-D-Gluc-1,4-(1-methoxyphenyl)Gal
C-terminal deletion mutant, pH 7.4, 37°C
0.043 - 0.61
o-nitrophenyl-alpha-N-acetylglucosaminide
-
0.23-0.25 in the presence of 0.025% bovine serum albumin
0.08
o-nitrophenyl-alpha-N-acetylglucosaminide
-
0.08 partially purified enzyme
0.13 - 0.2
p-nitrophenyl-alpha-N-acetylglucosaminide
-
-
0.13 - 0.2
p-nitrophenyl-alpha-N-acetylglucosaminide
-
0.21-0.55 in the presence of 0.025% bovine serum albumin
0.3
p-nitrophenyl-alpha-N-acetylglucosaminide
-
partially purified enzyme
0.12 - 1.6
phenyl-alpha-N-acetylglucosaminide
-
-
0.12 - 1.6
phenyl-alpha-N-acetylglucosaminide
-
1 M citrate buffer, pH 4.5
0.12 - 1.6
phenyl-alpha-N-acetylglucosaminide
-
form I 0.32, form II 0.29
0.12 - 1.6
phenyl-alpha-N-acetylglucosaminide
-
0.46-0.66 in the presence of 0.025% bovine serum albumin
1.4
phenyl-alpha-N-acetylglucosaminide
-
partially purified enzyme
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Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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