3.1.6.13: iduronate-2-sulfatase
This is an abbreviated version!
For detailed information about iduronate-2-sulfatase, go to the full flat file.
Word Map on EC 3.1.6.13
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3.1.6.13
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mucopolysaccharidosis
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lysosomal
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glycosaminoglycans
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x-linked
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dermatan
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heparan
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multisystemic
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sulfatases
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hepatosplenomegaly
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medicine
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arylsulfatase
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mpsii
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x-chromosome
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enzyme-replacement
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analysis
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synthesis
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alpha-l-iduronidase
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neuronopathic
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infusion-related
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drug development
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6-minute
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dysostosis
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diagnostics
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shire
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hurler
- 3.1.6.13
- mucopolysaccharidosis
- lysosomal
- glycosaminoglycans
-
x-linked
- dermatan
- heparan
-
multisystemic
-
sulfatases
-
hepatosplenomegaly
- medicine
- arylsulfatase
-
mpsii
-
x-chromosome
-
enzyme-replacement
- analysis
- synthesis
- alpha-l-iduronidase
-
neuronopathic
-
infusion-related
- drug development
-
6-minute
- dysostosis
- diagnostics
-
shire
- hurler
Reaction
Synonyms
2-sulfo-L-iduronate 2-sulfatase, chondroitinsulfatase, elaprase, Hunter corrective factor, I2S, IDS, IDS-Like, IDS-like enzyme, iduronate 2-sulfatase, iduronate 2-sulfate sulfatase-like, iduronate 2-sulfate-like enzyme, iduronate sulfatase, iduronate sulfate sulfatase, iduronate-2-sulfatase, iduronate-2-sulfate sulfatase, iduronate-2-sulphatase, iduronide-2-sulfate sulfatase, idurono-2-sulfatase, idursulfase, L-iduronate 2-sulfate sulfatase, L-idurono sulfate sulfatase, sulfatase, L-idurono-, sulfo-L-iduronate sulfatase, sulfoiduronate sulfohydrolase
ECTree
3.1.6.13 iduronate-2-sulfataseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 3.1.6.13 - iduronate-2-sulfatase
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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-alpha-idopyranosiduronic acid 2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronate + sulfate
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-
-
?
4-methylumbelliferyl-alpha-iduronate 2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronate + sulfate
4-methylumbelliferyl-alpha-iduronide 2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
-
-
-
?
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
4-methylumbelliferyl-alpha-L-iduronate 2-sulfate + H2O
4-methylumbelliferol + L-iduronate 2-sulfate
4-methylumbelliferyl-alpha-L-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-L-iduronide + sulfate
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-
-
-
?
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol + H2O
O-(alpha-L-idopyranosyluronic acid)-(1-4)-2,5-anhydro-D-mannitol + sulfate
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-
-
-
?
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate + H2O
O-(alpha-L-idopyranosyluronic acid)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate + sulfate
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-
-
-
?
O-(alpha-L-idopyranosyluronic acid-2-sulfate)-(1-4)-2,5 anhydromannose-6-sulfate
?
-
-
-
?
O-(alpha-L-iduronic acid 2-sulfate)-(1-3)-2,5-anhydro-D-talitol 4-sulfate + H2O
O-(alpha-L-iduronic acid)-(1-3)-2,5-anhydro-D-talitol 4-sulfate + sulfate
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-
-
-
?
O-(alpha-L-iduronic acid 2-sulfate)-(1-4)-D-O-(alpha 2-sulfaminoglucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid 2-sulfate)-D-O-2,5-anhydro-mannitol 6-sulfate + H2O
O-(alpha-L-iduronic acid)-(1-4)-D-O-(alpha 2-sulfaminoglucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid)-D-O-2,5-anhydro-mannitol 6-sulfate + sulfate
-
-
-
-
?
O-(alpha-L-iduronic acid 2-sulfate)-(1-4)-D-O-(alpha 2-sulfaminoglucosamine 6-sulfate)-(1-4)-O-(beta-D-glucuronic acid or L-iduronic acid)-(1-4)-D-O-(alpha-N-acetylglucosamine)-(1-3)-D-O-gulonic acid + H2O
O-(alpha-L-iduronic acid)-(1-4)-D-O-(alpha 2-sulfaminoglucosamine 6-sulfate)-(1-4)-O-(beta-D-glucuronic acid or L-iduronic acid)-(1-4)-D-O-(alpha-N-acetylglucosamine)-(1-3)-D-O-gulonic acid + sulfate
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-
-
-
?
O-(alpha-L-iduronic acid 2-sulfate)-(1-4)-D-O-(alpha glucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid 2-sulfate)-D-O-2,5-anhydro-mannitol 6-sulfate + H2O
O-(alpha-L-iduronic acid)-(1-4)-D-O-(alpha glucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid)-D-O-2,5-anhydro-mannitol 6-sulfate + sulfate
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-
-
-
?
O-(alpha-L-iduronic acid 2-sulfate)-(1-4)-D-O-(alpha-N-acetylglucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid 2-sulfate)-D-O-2,5-anhydro-mannitol 6-sulfate + H2O
O-(alpha-L-iduronic acid)-(1-4)-D-O (alpha N-acetylglucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid)-D-O-2,5-anhydro-mannitol 6-sulfate + sulfate
-
-
-
-
?
4-methylumbelliferyl-alpha-iduronate 2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronate + sulfate
-
-
-
-
?
4-methylumbelliferyl-alpha-iduronate 2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronate + sulfate
-
-
-
?
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
-
-
-
?
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
-
-
-
-
?
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
-
-
-
-
?
4-methylumbelliferyl-alpha-L-iduronate 2-sulfate + H2O
4-methylumbelliferol + L-iduronate 2-sulfate
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-
-
?
4-methylumbelliferyl-alpha-L-iduronate 2-sulfate + H2O
4-methylumbelliferol + L-iduronate 2-sulfate
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artificial substrate
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-
?
dermatan sulfate + H2O
dermatan + sulfate
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135566, 135567, 135568, 135569, 135570, 135571, 135573, 135574, 135575, 135576, 135577, 135578, 691866
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-
?
heparan sulfate + H2O
dermatan + sulfate
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-
135566, 135567, 135568, 135569, 135570, 135571, 135573, 135574, 135575, 135576, 135577, 135578, 691866
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-
?
heparin + H2O
desulfated heparin + sulfate
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-
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?
additional information
?
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the enzyme is involved in the catabolism of the mucopolysaccharides heparan and dermatan sulfate
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?
additional information
?
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the enzyme is involved in the sequential degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate
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?
additional information
?
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the enzyme is responsible for heparin sulfate and dermatan sulfate degradation
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?
additional information
?
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the enzyme is responsible for heparin sulfate and dermatan sulfate degradation
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?
additional information
?
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involved in the degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate. An enzyme deficiency causes the lysosomal storage disorder muco-polysaccharidosis type II
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-
?
additional information
?
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involved in the degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate. An enzyme deficiency causes the lysosomal storage disorder muco-polysaccharidosis type II
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-
?
additional information
?
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mucopolysaccharidosis type II is a lysosomal storage disorder related to a deficiency in the enzyme iduronate-2-sulfatase
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-
?
additional information
?
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mechanism follows a SN2 nucleophilic attack of the formylglycine FGH84 on the sulfur atom to generate the covalent formylglycine-S adduct, followed by elimination of bound sulfate and rehydration of the resultant formylglycine aldehyde. Residues H229, H138, D334 and K347 are likely to play active roles in proton transfer or activation of water during the reaction cycle
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-
?
additional information
?
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mechanism follows a SN2 nucleophilic attack of the formylglycine FGH84 on the sulfur atom to generate the covalent formylglycine-S adduct, followed by elimination of bound sulfate and rehydration of the resultant formylglycine aldehyde. Residues H229, H138, D334 and K347 are likely to play active roles in proton transfer or activation of water during the reaction cycle
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-
?
additional information
?
-
-
The enzyme has a potential role in normal pathway of lysosomal degradation of secretory peptides and is likely to be essential to maintain pancreatic beta-cell function
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?
additional information
?
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the enzyme is involved in the catabolism of the mucopolysaccharides heparan and dermatan sulfate
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?
