3.1.6.4: N-acetylgalactosamine-6-sulfatase
This is an abbreviated version!
For detailed information about N-acetylgalactosamine-6-sulfatase, go to the full flat file.
Word Map on EC 3.1.6.4
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3.1.6.4
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mucopolysaccharidosis
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lysosomal
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morquio
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keratan
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glycosaminoglycans
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dysplasia
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chondroitin-6-sulfate
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sulfatases
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arylsulfatase
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elosulfase
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n-acetylgalactosamine-4-sulfatase
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medicine
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kyphoscoliosis
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odontoid
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sumf1
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synthesis
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sanfilippo
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pectus
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platyspondyly
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carinatum
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valgum
- 3.1.6.4
- mucopolysaccharidosis
- lysosomal
- morquio
- keratan
- glycosaminoglycans
- dysplasia
- chondroitin-6-sulfate
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sulfatases
- arylsulfatase
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elosulfase
- n-acetylgalactosamine-4-sulfatase
- medicine
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kyphoscoliosis
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odontoid
- sumf1
- synthesis
-
sanfilippo
-
pectus
-
platyspondyly
-
carinatum
-
valgum
Reaction
Synonyms
6-sulfatase, acetylgalactosamine 6-sulfatase, AtsA2, chondroitin sulfatase, chondroitinase, chondroitinsulfatase, galactose-6-sulfatase, galactose-6-sulfate sulfatase, GalNAc6S sulfatase, GALNS, N-acetylgalactosamine 6-sulfatase, N-acetylgalactosamine 6-sulphate sulphatase, N-acetylgalactosamine-6-sulfatase, N-acetylgalactosamine-6-sulfate sulfatase, sulfatase, acetylgalactosamine 6-, sulfatase, chondroitin
ECTree
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Substrates Products
Substrates Products on EC 3.1.6.4 - N-acetylgalactosamine-6-sulfatase
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REACTION DIAGRAM
4-methylumbelliferyl beta-D-galactoside 6-sulfate + H2O
4-methylumbelliferyl beta-D-galactoside + sulfate
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-
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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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-
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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
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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 + 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 + 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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-
-
-
?
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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?
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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-
?
sulfate + UDP-N-acetylgalactosamine
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?
UDP-N-acetylgalactosamine 6-sulfate + H2O
sulfate + UDP-N-acetylgalactosamine
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-
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?
?
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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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?