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Information on EC 3.1.6.4 - N-acetylgalactosamine-6-sulfatase
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EC Tree
3.1.6.4 N-acetylgalactosamine-6-sulfataseSpecify your search results
Word Map
- 3.1.6.4
- mucopolysaccharidosis
- lysosomal
- morquio
- keratan
- glycosaminoglycans
- dysplasia
- chondroitin-6-sulfate
-
sulfatases
- arylsulfatase
-
elosulfase
- n-acetylgalactosamine-4-sulfatase
- medicine
-
kyphoscoliosis
-
odontoid
- sumf1
- synthesis
-
sanfilippo
-
pectus
-
platyspondyly
-
carinatum
-
valgum
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Synonyms
galns, n-acetylgalactosamine-6-sulfate sulfatase, n-acetylgalactosamine-6-sulfatase, 6-sulfatase, chondroitin sulfatase, n-acetylgalactosamine 6-sulfatase, galactose-6-sulfatase, galactose-6-sulfate sulfatase, n-acetylgalactosamine 6-sulphate sulphatase, atsa2, 
more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
acetylgalactosamine 6-sulfatase
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-
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AtsA2
chondroitin sulfatase
chondroitinase
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chondroitinsulfatase
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galactose-6-sulfate sulfatase
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GalNAc6S sulfatase
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GALNS
N-acetylgalactosamine 6-sulfatase
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N-acetylgalactosamine 6-sulphate sulphatase
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N-acetylgalactosamine-6-sulfate sulfatase
sulfatase, acetylgalactosamine 6-
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sulfatase, chondroitin
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chondroitin sulfatase
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GALNS
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-
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N-acetylgalactosamine-6-sulfate sulfatase
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-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
D-GalNAc-6-sulfate + H2O = D-GalNAc + sulfate
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-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
hydrolysis of sulfuric ester
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-
-
-
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PATHWAY SOURCE
PATHWAYS
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SYSTEMATIC NAME
IUBMB Comments
N-acetyl-D-galactosamine-6-sulfate 6-sulfohydrolase
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CAS REGISTRY NUMBER
COMMENTARY
59299-00-2
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9025-60-9
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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
4-methylumbelliferyl beta-D-galactoside 6-sulfate + H2O
4-methylumbelliferyl beta-D-galactoside + sulfate
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-
-
?
4-methylumbelliferyl-beta-D-galactopyranoside-6-sulfate + H2O
4-methylumbelliferyl-beta-D-galactopyranoside + sulfate
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-
-
-
?
4-methylumbelliferyl-beta-D-galactose-6-sulfate + H2O
4-methylumbelliferyl-beta-D-galactose + sulfate
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-
-
-
?
4-methylumbelliferyl-beta-D-galactoside-6-sulfate + H2O
4-methylumbelliferyl-beta-D-galactoside + sulfate
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-
-
?
beta-N-acetyl-D-galactosamine-6-sulfate-(1-4)-beta-D-glucuronic acid-(1-3)-N-acetyl-D-galactosaminitol 6-sulfate + H2O
sulfate + ?
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-
-
-
?
Gal(6-SO4)beta(1-4)-GlcAc(6-SO4)beta(1-4)-D-galactitol + H2O
sulfate + ?
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-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
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in chondroitin sulfate or keratan sulfate, decreased enzymatic activity in fasting rats
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?
N-acetylgalactosamine 6-sulfate-beta(1-4)glucuronic acid-beta(1-3)-N-acetylgalactosaminitol 6-sulfate + H2O
sulfate + ?
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sulfate is hydrolyzed only from the non-reducing terminal
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-
?
O-(beta-D-6-sulfo-2-acetamido-2-deoxygalactosyl)-(1-4)-O-beta-D-glucuronosyl-(1-3)-O-beta-D-6-sulfo-2-acetamido-2-deoxy-galactitol + H2O
sulfate + ?
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-
-
-
?
O-(beta-D-sulfogalactosyl)-(1-4)-1,5-anhydro-D-mannitol 6-sulfate + H2O
?
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?
sulfated tetrasaccharide + H2O
?
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sulfated tetrasaccharide obtained by digestion of purified chondroitin 6-sulfate with resticular hyaluronidase
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?
chondroitin sulfate + H2O
chondroitin + sulfate
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cleaves sulfate from the non-reducing N-acetylgalactosamine 6-sulfate end group
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-
?
chondroitin sulfate + H2O
chondroitin + sulfate
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cleaves sulfate from the non-reducing N-acetylgalactosamine 6-sulfate end group
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-
?
chondroitin sulfate + H2O
chondroitin + sulfate
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in chondroitin sulfate or keratan sulfate
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?
chondroitin-6-sulfate + H2O
chondroitin + sulfate
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6-sulfated tetrasaccharide from chondroitin-6-sulfate
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?
galactose 6-sulfate + H2O
galactose + sulfate
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about 40% of the activity with N-acetylgalactosamine 6-sulfate
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?
galactose 6-sulfate + H2O
galactose + sulfate
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about 40% of the activity with N-acetylgalactosamine 6-sulfate
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?
galactose 6-sulfate + H2O
galactose + sulfate
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in chondroitin sulfate or keratan sulfate
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?
galactose 6-sulfate + H2O
galactose + sulfate
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in chondroitin sulfate or keratan sulfate
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?
galactose 6-sulfate + H2O
galactose + sulfate
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in chondroitin sulfate or keratan sulfate
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?
N-acetylgalactosamine 6-sulfate + H2O
N-acetylgalactosamine + sulfate
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?
N-acetylgalactosamine 6-sulfate + H2O
N-acetylgalactosamine + sulfate
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?
N-acetylgalactosamine 6-sulfate + H2O
N-acetylgalactosamine + sulfate
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?
tetrasaccharide trisulfate + H2O
sulfate + N-acetylglucosamine + galactose
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bearing the non-reducing N-acetylglucosamine 6-sulfate
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?
tetrasaccharide trisulfate + H2O
sulfate + N-acetylglucosamine + galactose
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bearing the non-reducing N-acetylglucosamine 6-sulfate
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?
trisaccharide disulfate + H2O
sulfate + ?
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trisaccharide disulfate prepared from keratan sulfate by sequential degradation with endo-beta-galactosidase, N-acetylglucosamine-6-sulfatase and exo-beta-N-acetylglucosanminidase
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?
trisaccharide disulfate + H2O
sulfate + ?
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trisaccharide disulfate prepared from keratan sulfate by sequential degradation with endo-beta-galactosidase, N-acetylglucosamine-6-sulfatase and exo-beta-N-acetylglucosanminidase
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?
UDP-N-acetylgalactosamine 6-sulfate + H2O
sulfate + UDP-N-acetylgalactosamine
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?
UDP-N-acetylgalactosamine 6-sulfate + H2O
sulfate + UDP-N-acetylgalactosamine
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?
additional information
?
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enzyme is involved in chondroitin sulfate breakdown
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?
additional information
?
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concerted action of exoglycosidases and EC 3.1.6.4 in degradation of polymeric chondroitin 4-sulfate, no contribution of the enzyme to the degradation of chondroitin
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?
additional information
?
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does not hydrolyze sulfate from N-acetylglucosamine 6-sulfate
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?
additional information
?
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enzyme is involved in lysosomal degradation of the glycosaminoglycans keratan sulfate and chondroitin 6-sulfate
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?
additional information
?
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the enzyme catalyzes the first step of intralysosomal keratan sulfate catabolism. In Morquito A syndrome the N-acetylglucosamine 6-sulfate sulfatase deficiency causes the accumulation of keratan sulfate in tissue and results in generalized skeletal dysplasia in affected patients
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?
additional information
?
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enzyme deficiency causes mucopolysaccharidosis type IV, i.e. Morquio A syndrome, an autosomal recessive inborn error, due to accumulation of sulfated glycosaminoglycans in lysosomes, overview
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?
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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
additional information
?
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enzyme is involved in chondroitin sulfate breakdown
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?
additional information
?
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concerted action of exoglycosidases and EC 3.1.6.4 in degradation of polymeric chondroitin 4-sulfate, no contribution of the enzyme to the degradation of chondroitin
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?
additional information
?
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enzyme is involved in lysosomal degradation of the glycosaminoglycans keratan sulfate and chondroitin 6-sulfate
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?
additional information
?
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the enzyme catalyzes the first step of intralysosomal keratan sulfate catabolism. In Morquito A syndrome the N-acetylglucosamine 6-sulfate sulfatase deficiency causes the accumulation of keratan sulfate in tissue and results in generalized skeletal dysplasia in affected patients
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-
?
additional information
?
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enzyme deficiency causes mucopolysaccharidosis type IV, i.e. Morquio A syndrome, an autosomal recessive inborn error, due to accumulation of sulfated glycosaminoglycans in lysosomes, overview
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?
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
1,4-dideoxy-1,4-imino-D-arabinitol-gold
gold glyconanoparticles decorated with the natural iminosugar 1,4-dideoxy-1,4-imino-D-arabinitol, i.e. DAB-1 strongly and selectively inhibit N-acetylgalactosamine-6-sulfatase. The strategy employed allowed the enhancement of the multivalent presentation of the iminosugar onto the surface of gold nanoparticles
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
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cotransfection with sulfatase modifying factor 1 shows an increment up to 2.6fold in GALNS activity, cells cotransfected with elongation factor 1alpha show GALNS activity continued to increase until day 8 postransfection
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.12
N-acetylgalactosamine 6-sulfate-beta(1-4)glucuronic acid-beta(1-3)-N-acetylgalactosaminitol 6-sulfate
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0.012 - 0.015
O-(beta-D-6-sulfo-2-acetamido-2-deoxygalactosyl)-(1-4)-O-beta-D-glucuronosyl-(1-3)-O-beta-D-6-sulfo-2-acetamido-2-deoxy-galactitol
0.012
O-(beta-D-6-sulfo-2-acetamido-2-deoxygalactosyl)-(1-4)-O-beta-D-glucuronosyl-(1-3)-O-beta-D-6-sulfo-2-acetamido-2-deoxy-galactitol
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liver enzyme
0.015
O-(beta-D-6-sulfo-2-acetamido-2-deoxygalactosyl)-(1-4)-O-beta-D-glucuronosyl-(1-3)-O-beta-D-6-sulfo-2-acetamido-2-deoxy-galactitol
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recombinant enzyme
0.05
O-(beta-D-sulfogalactosyl)-(1-4)-1,5-anhydro-D-mannitol 6-sulfate
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liver enzyme
0.096
O-(beta-D-sulfogalactosyl)-(1-4)-1,5-anhydro-D-mannitol 6-sulfate
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recombinant enzyme
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IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
3.5 - 4
5.6
6.5
-
release of sulfate from galactose 6-sulfate and N-acetylgalactosamine 6-sulfate
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pH RANGE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
3.3 - 4.2
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pH 3.0: about 45% of maximal activity, pH 4.2: about 55% of maximal activity, hydrolysis of N-acetylgalactosamine 6-sulfate, 0.02 M acetate buffer
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
isolated from humans, a penguin, an armadillo and dog food
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brenda
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UniProt
brenda
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
in post-natal ventral rat prostate, a distinct and reciprocal localization of arylsulfatase B and N-acetylgalactosamine-6-sulfatase is seen, with arylsulfatase B predominant in the stroma and N-acetylgalactosamine-6-sulfatase predominant in the epithelium. Estrogen treatment does not inhibit the increase in GALNS activity between days 5 and 30
brenda
no detectable enzyme amount in Mucopolysaccheridosis type IVA patients
brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
metabolism
modification of expression of the enzyme regulates the content of chondroitin sulfate
physiological function
malfunction
mucopolysaccharidosis IVA, MPS IVA, is an autosomal recessive disorder caused by N-acetylgalactosamine-6-sulfate sulfatase deficiency and leading to lysosomal accumulation of the glycosaminoglycans keratan sulfate and chondroitin-6-sulfate
malfunction
deficiencies of N-acetylgalactosamine-6-sulfatase is associated with the mucopolysaccharidoses
physiological function
in post-natal ventral rat prostate, a distinct and reciprocal localization of arylsulfatase B and N-acetylgalactosamine-6-sulfatase is seen, with arylsulfatase B predominant in the stroma and N-acetylgalactosamine-6-sulfatase predominant in the epithelium. Estrogen treatment does not inhibit the increase in GALNS activity between days 5 and 30
physiological function
in prostate stem cells, when N-acetylgalactosamine-4-sulfatase ARSB is reduced by silencing or galactosamine-N-acetyl-6-sulfatase GALNS is increased by overexpression, activity of non-receptor tyrosine phosphatase SHP2 declines, attributable to increased binding of SHP2 with C4S. This leads to increases in phospho-ERK1/2, Myc/Max nuclear DNA binding, DNA methyltransferase (DNMT) activity and expression, and methylation of the Dickkopf Wnt signaling pathway inhibitor (DKK)3 promoter and to reduced DKK3 expression. Since DKK3 negatively regulates Wnt/beta-catenin signaling, silencing of ARSB or overexpression of GALNS increases Wnt/beta-catenin signaling
physiological function
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strain is able to grow on N-acetylglucosamine-6-sulfate, while N-acetylglucosamine-3-sulfate, N-acetylgalactosamine-3-sulfate, and N-acetylgalactosamine-6-sulfate do not support appreciable growth. Gene cluster Ats2 is required for N-acetylglucosamine-6-sulfate metabolism
physiological function
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strain is able to grow on N-acetylglucosamine-6-sulfate, while N-acetylglucosamine-3-sulfate, N-acetylgalactosamine-3-sulfate, and N-acetylgalactosamine-6-sulfate do not support appreciable growth. Gene cluster Ats2 is required for N-acetylglucosamine-6-sulfate metabolism
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