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Information on EC 3.1.6.12 - N-acetylgalactosamine-4-sulfatase
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EC Tree
3.1.6.12 N-acetylgalactosamine-4-sulfataseIUBMB Comments
Acts also on N-acetylglucosamine 4-sulfate.
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Word Map
- 3.1.6.12
- mucopolysaccharidosis
- lysosomal
-
maroteaux-lamy
- glycosaminoglycans
- arylsulfatase
- dermatan
- feline
-
rhasb
- medicine
-
galsulfase
-
sulfatases
-
stair
-
enzyme-replacement
-
mps-vi
- dysostosis
- diagnostics
- nutrition
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota
Synonyms
n-acetylgalactosamine-4-sulfatase, 4-sulfatase, 4-sulphatase, n-acetylgalactosamine 4-sulfatase, n-acetylgalactosamine-4-sulphatase, chondrosulfatase, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
acetylgalactosamine 4-sulfatase
-
-
-
-
ARSB
arylsulfatase B
ASB
chondroitinase
-
-
-
-
chondroitinsulfatase
-
-
-
-
G4S
-
-
-
-
N-acetylgalactosamine 4-sulfatase
N-acetylgalactosamine 4-sulfate sulfohydrolase
-
-
-
-
N-acetylgalactosamine-4-sulfatase
sulfatase, acetylgalactosamine 4-
-
-
-
-
arylsulfatase B
-
-
-
-
arylsulfatase B
-
-
ASB
-
-
-
-
N-acetylgalactosamine-4-sulfatase
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
D-GalNAc-4-sulfate + H2O = sulfate + D-GalNAc
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
hydrolysis of sulfuric ester
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
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SYSTEMATIC NAME
IUBMB Comments
N-acetyl-D-galactosamine-4-sulfate 4-sulfohydrolase
Acts also on N-acetylglucosamine 4-sulfate.
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CAS REGISTRY NUMBER
COMMENTARY
55354-43-3
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(2-acetamido-2-deoxy-O-(beta-D-glucuronic acid)-4-O-sulpho-D-galactose)2 + H2O
(2-acetamido-2-deoxy-O-(beta-D-glucuronic acid)-D-galactose)2 + sulfate
-
exo-sulfatase, stepwise degradation of glycosaminoglycans
-
-
?
4-methylumbelliferyl sulfate + H2O
sulfate + 4-methylumbelliferone
-
-
-
?
carbobenzoxyglucosamine 4,6-disulfate + H2O
carbobenzoxyglucosamine 6-sulfate + sulfate
-
-
-
-
?
chondroitin 4-sulfate-heptasaccharide + H2O
chondroitin 4-sulfate-heptasaccharide + sulfate
chondroitin 4-sulfate-tetrasaccharide + H2O
chondroitin 4-sulfate-tetrasaccharide + sulfate
-
only the 4-sulfated endgroup is attacked by the enzyme, sulfate is released only in presence of beta-glucuronidase, acts as exosulphatase
-
-
?
chondroitin-4-sulfate + H2O
chondroitin + sulfate
-
arylsulfatase B removes 4-sulfate groups from the sulfated glycosaminoglycans chondroitin-4-sulfate and dermatan sulfate
-
-
?
N-acetygalactosamine 4-sulfate-(1-4)-beta-glucuronic acid-(1-3)-beta-N-acetylgalactosaminitol 4-sulfate + H2O
N-acetygalactosamine-(1-4)-beta glucuronic acid-(1-3)-beta N-acetylgalactosaminitol 4-sulfate + sulfate
N-acetylgalactosamine 4,6-bisulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)3 + H2O
N-acetylgalactosamine 6-sulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine)3 + sulfate
-
hydrolysis only at the nonreducing residues
-
-
?
N-acetylgalactosamine 4-sulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)2 + H2O
N-acetylgalactosamine-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)2 + sulfate
-
only the 4-sulfated endgroup is attacked by the enzyme
-
-
?
N-acetylgalactosamine 4-sulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)3 + H2O
N-acetylgalactosamine-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine)3 + sulfate
-
only the 4-sulfated endgroup is attacked by the enzyme
-
-
?
N-acetylgalactosamine 4-sulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)4 + H2O
N-acetylgalactosamine-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)4 + sulfate
-
only the 4-sulfated endgroup is attacked by the enzyme
-
-
?
N-acetylgalactosamine 4-sulfate-beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate + H2O
N-acetylgalactosamine-beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate + sulfate
-
-
-
-
?
N-acetylglucosamine 4-sulfate + H2O
N-acetylglucosamine + sulfate
-
-
-
-
?
UDP-N-acetylgalactosamine 4,6-bisulfate + H2O
UDP-N-acetylgalactosamine 6-sulfate + sulfate
-
-
-
-
?
4-methylumbelliferyl sulfate + H2O
4-methylumbelliferol + sulfate
-
-
-
?
4-methylumbelliferyl sulfate + H2O
4-methylumbelliferone + sulfate
-
-
-
-
?
4-methylumbelliferyl sulfate + H2O
4-methylumbelliferone + sulfate
-
-
-
-
?
4-methylumbelliferyl sulfate + H2O
4-methylumbelliferone + sulfate
-
-
-
?
chondroitin 4-sulfate + H2O
chondroitin + sulfate
-
-
-
-
?
chondroitin 4-sulfate-heptasaccharide + H2O
chondroitin 4-sulfate-heptasaccharide + sulfate
-
reduced activity in patients with Maroteaux-Lamy Syndrome
-
-
?
chondroitin 4-sulfate-heptasaccharide + H2O
chondroitin 4-sulfate-heptasaccharide + sulfate
-
-
-
-
?
chondroitin 4-sulfate-heptasaccharide + H2O
chondroitin 4-sulfate-heptasaccharide + sulfate
-
-
-
-
?
dermatan sulfate + H2O
dermatan + sulfate
-
reduced activity in patients with Maroteaux-Lamy Syndrome
-
-
?
dermatan sulfate + H2O
dermatan + sulfate
-
arylsulfatase B removes 4-sulfate groups from the sulfated glycosaminoglycans chondroitin-4-sulfate and dermatan sulfate
-
-
?
N-acetygalactosamine 4-sulfate-(1-4)-beta-glucuronic acid-(1-3)-beta-N-acetylgalactosaminitol 4-sulfate + H2O
N-acetygalactosamine-(1-4)-beta glucuronic acid-(1-3)-beta N-acetylgalactosaminitol 4-sulfate + sulfate
-
-
-
-
?
N-acetygalactosamine 4-sulfate-(1-4)-beta-glucuronic acid-(1-3)-beta-N-acetylgalactosaminitol 4-sulfate + H2O
N-acetygalactosamine-(1-4)-beta glucuronic acid-(1-3)-beta N-acetylgalactosaminitol 4-sulfate + sulfate
-
oligosaccharides
-
-
?
N-acetyl-D-galactosamine 4-sulfate + H2O
N-acetyl-D-galactosamine + sulfate
-
-
-
-
?
N-acetyl-D-galactosamine 4-sulfate + H2O
N-acetyl-D-galactosamine + sulfate
-
-
-
-
?
N-acetyl-D-galactosamine 4-sulfate + H2O
N-acetyl-D-galactosamine + sulfate
-
-
-
-
?
N-acetyl-D-galactosamine 4-sulfate + H2O
N-acetyl-D-galactosamine + sulfate
-
-
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
weak iduronate sulfatase activity
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
no activity on galactosamine 6-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
p-nitrocatechol sulfate + H2O
p-nitrocatechol + sulfate
-
-
-
-
?
p-nitrocatechol sulfate + H2O
p-nitrocatechol + sulfate
-
enzymes catalyze in addition to reaction of EC 3.1.6.12 also arylsulfatase reaction , see also EC 3.1.6.1
-
-
?
p-nitrocatechol sulfate + H2O
p-nitrocatechol + sulfate
-
enzymes catalyze in addition to reaction of EC 3.1.6.12 also arylsulfatase reaction , see also EC 3.1.6.1
-
-
?
p-nitrocatechol sulfate + H2O
p-nitrocatechol + sulfate
-
enzymes catalyze in addition to reaction of EC 3.1.6.12 also arylsulfatase reaction , see also EC 3.1.6.1
-
-
?
p-nitrocatechol sulfate + H2O
p-nitrocatechol + sulfate
-
enzymes catalyze in addition to reaction of EC 3.1.6.12 also arylsulfatase reaction , see also EC 3.1.6.1
-
-
?
UDP-N-acetylgalactosamine-4-sulfate + H2O
UDP-N-acetylgalactosamine + sulfate
-
-
-
-
?
UDP-N-acetylgalactosamine-4-sulfate + H2O
UDP-N-acetylgalactosamine + sulfate
-
-
-
-
?
UDP-N-acetylgalactosamine-4-sulfate + H2O
UDP-N-acetylgalactosamine + sulfate
-
-
-
-
?
UDP-N-acetylgalactosamine-4-sulfate + H2O
UDP-N-acetylgalactosamine + sulfate
-
-
-
-
?
additional information
?
-
mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-4-sulfatase. Mutations in the N-acetylgalactosamine-4-sulfatase gene are responsible for 4S deficiency, which leads to the intralysosomal storage of partially degraded glycosaminoglycan, dermatan sulfate, and chondroitin 4-sulfate
-
-
?
additional information
?
-
-
mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-4-sulfatase. Mutations in the N-acetylgalactosamine-4-sulfatase gene are responsible for 4S deficiency, which leads to the intralysosomal storage of partially degraded glycosaminoglycan, dermatan sulfate, and chondroitin 4-sulfate
-
-
?
additional information
?
-
-
the enzyme is required for the degradation of the glycosaminoglycan substrates dermatan and chondroitin sulfate. A 4-sulfatase deficiency results in the accumulation of undegraded substrate and causes the severe lysosomal storage disorder mucopolysaccharidosis type VI or Maroteaux-Lamy syndrome. A wide variation in clinical severity is observed between MPS VI patients and reflects the number of different 4-sulfatase mutations that can cause the disorder.Y210C is detected in about 10% of the MPS VI patients
-
-
?
additional information
?
-
-
ARSB removes 4-sulfate groups from the nonreducing end of chondroitin-4-sulfate and dermatan sulfate
-
-
?
additional information
?
-
-
ASB is a lysosomal exohydrolase, cleaving the 4-sulfate from the N-acetylgalactosamine-4-sulfate residue at the nonreducing terminal of glycosaminoglycan structures
-
-
?
additional information
?
-
-
ARSB removes 4-sulfate groups from the nonreducing end of chondroitin-4-sulfate and dermatan sulfate
-
-
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
chondroitin-4-sulfate + H2O
chondroitin + sulfate
-
arylsulfatase B removes 4-sulfate groups from the sulfated glycosaminoglycans chondroitin-4-sulfate and dermatan sulfate
-
-
?
dermatan sulfate + H2O
dermatan + sulfate
-
arylsulfatase B removes 4-sulfate groups from the sulfated glycosaminoglycans chondroitin-4-sulfate and dermatan sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
weak iduronate sulfatase activity
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
no activity on galactosamine 6-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
N-acetyl-D-galactosamine 4-sulfate units + H2O
N-acetyl-D-galactosamine units + sulfate
-
acts also on N-acetylglucosamine 4-sulfate
-
-
?
additional information
?
-
mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-4-sulfatase. Mutations in the N-acetylgalactosamine-4-sulfatase gene are responsible for 4S deficiency, which leads to the intralysosomal storage of partially degraded glycosaminoglycan, dermatan sulfate, and chondroitin 4-sulfate
-
-
?
additional information
?
-
-
mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-4-sulfatase. Mutations in the N-acetylgalactosamine-4-sulfatase gene are responsible for 4S deficiency, which leads to the intralysosomal storage of partially degraded glycosaminoglycan, dermatan sulfate, and chondroitin 4-sulfate
-
-
?
additional information
?
-
-
the enzyme is required for the degradation of the glycosaminoglycan substrates dermatan and chondroitin sulfate. A 4-sulfatase deficiency results in the accumulation of undegraded substrate and causes the severe lysosomal storage disorder mucopolysaccharidosis type VI or Maroteaux-Lamy syndrome. A wide variation in clinical severity is observed between MPS VI patients and reflects the number of different 4-sulfatase mutations that can cause the disorder.Y210C is detected in about 10% of the MPS VI patients
-
-
?
additional information
?
-
-
ARSB removes 4-sulfate groups from the nonreducing end of chondroitin-4-sulfate and dermatan sulfate
-
-
?
additional information
?
-
-
ASB is a lysosomal exohydrolase, cleaving the 4-sulfate from the N-acetylgalactosamine-4-sulfate residue at the nonreducing terminal of glycosaminoglycan structures
-
-
?
additional information
?
-
-
ARSB removes 4-sulfate groups from the nonreducing end of chondroitin-4-sulfate and dermatan sulfate
-
-
?
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
chloride
Mn2+
phosphate
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
ethanol
astrocyte treatment with ethanol inhibits the activity of arylsulfatase B, and triggers the degradation of chondroitin-4-sulfate, increases total sulfated glycosaminoglycans, chondroitin-4-sulfate and neurocan core-protein content and inhibits neurite outgrowth in neurons cocultured with ethanol-treated astrocytes in vitro. Ethanol also inhibits arylsulfatase B activity and increases sulfated glycosaminoglycans and neurocan levels in the developing hippocampus after in vivo ethanol exposure
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
vanadate
maximal activation achieved for mutant arylsulfatase B C91S at highest vanadate concentration of 0.4 mM with concomitant irradation for 120 min
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.03
N-acetylgalactosamine 4-sulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)2
-
-
0.049
N-acetylgalactosamine 4-sulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)3
-
-
0.064
N-acetylgalactosamine 4-sulfate-(beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate)4
-
-
0.027
N-acetylgalactosamine 4-sulfate-beta-glucuronosyl-(1-3)-N-acetylgalactosamine 4-sulfate
-
-
3.2
p-nitrocatechol sulfate
-
pH 7.4, 37°C, enzyme from animals infected with Schistosoma mansoni
3.9
p-nitrocatechol sulfate
-
pH 7.4, 37°C, enzyme from animals without infection
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TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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