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Information on EC 3.1.6.13 - iduronate-2-sulfatase
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3.1.6.13 iduronate-2-sulfataseSpecify your search results
Word Map
- 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
The enzyme appears in viruses and cellular organisms
Synonyms
iduronate-2-sulfatase, idursulfase, iduronate sulfatase, iduronate 2-sulfatase, iduronate-2-sulphatase, iduronate-2-sulfate sulfatase, elaprase, 2-sulfo-l-iduronate 2-sulfatase, ids-like enzyme, iduronate sulfate sulfatase, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
2-sulfo-L-iduronate 2-sulfatase
-
-
-
-
chondroitinsulfatase
-
-
-
-
IDS
iduronate sulfatase
iduronate sulfate sulfatase
-
-
-
-
iduronate-2-sulfatase
iduronate-2-sulfate sulfatase
iduronide-2-sulfate sulfatase
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-
-
-
idurono-2-sulfatase
-
-
-
-
L-iduronate 2-sulfate sulfatase
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-
-
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L-idurono sulfate sulfatase
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-
-
-
sulfatase, L-idurono-
-
-
-
-
sulfo-L-iduronate sulfatase
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-
-
-
sulfoiduronate sulfohydrolase
-
-
-
-
iduronate sulfatase
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-
-
-
iduronate-2-sulfatase
-
-
-
-
iduronate-2-sulfatase
-
-
iduronate-2-sulfate sulfatase
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
L-iduronate-2-sulfate + H2O = L-iduronate + sulfate
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
hydrolysis of sulfuric ester
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-
-
-
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SYSTEMATIC NAME
IUBMB Comments
L-iduronate-2-sulfate 2-sulfohydrolase
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CAS REGISTRY NUMBER
COMMENTARY
50936-59-9
-
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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-alpha-idopyranosiduronic acid 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-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
-
-
-
-
?
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
-
-
-
-
?
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
-
-
-
-
?
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
-
-
-
-
?
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
-
-
-
-
?
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
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-
-
-
?
4-methylumbelliferyl-alpha-iduronate 2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronate + sulfate
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-
-
-
?
4-methylumbelliferyl-alpha-iduronate 2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronate + sulfate
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-
-
?
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
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-
-
?
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
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-
-
-
?
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
4-methylumbelliferyl-alpha-iduronide + sulfate
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-
-
-
?
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
-
-
?
dermatan sulfate + H2O
dermatan + sulfate
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135566, 135567, 135568, 135569, 135570, 135571, 135573, 135574, 135575, 135576, 135577, 135578, 691866
-
-
?
heparan sulfate + H2O
dermatan + sulfate
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-
135566, 135567, 135568, 135569, 135570, 135571, 135573, 135574, 135575, 135576, 135577, 135578, 691866
-
-
?
heparin + H2O
desulfated heparin + sulfate
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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
?
-
-
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
?
-
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
?
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-
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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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
dermatan sulfate + H2O
dermatan + sulfate
-
-
-
-
?
heparan sulfate + H2O
dermatan + sulfate
-
-
-
-
?
heparin + H2O
desulfated heparin + sulfate
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-
-
-
?
additional information
?
-
-
the enzyme is involved in the catabolism of the mucopolysaccharides heparan and dermatan sulfate
-
?
additional information
?
-
-
the enzyme is involved in the sequential degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate
-
?
additional information
?
-
-
the enzyme is responsible for heparin sulfate and dermatan sulfate degradation
-
?
additional information
?
-
-
the enzyme is responsible for heparin sulfate and dermatan sulfate degradation
-
?
additional information
?
-
involved in the degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate. An enzyme deficiency causes the lysosomal storage disorder muco-polysaccharidosis type II
-
-
?
additional information
?
-
-
involved in the degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate. An enzyme deficiency causes the lysosomal storage disorder muco-polysaccharidosis type II
-
-
?
additional information
?
-
-
mucopolysaccharidosis type II is a lysosomal storage disorder related to a deficiency in the enzyme iduronate-2-sulfatase
-
-
?
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
-
?
additional information
?
-
-
the enzyme is involved in the catabolism of the mucopolysaccharides heparan and dermatan sulfate
-
?
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
-
effect of IDS on insulin secretion does not result from increased intracellular Ca2+ signaling
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
N-actetylglucosamine 6-sulfate-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
competitive inhibitor
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol
-
competitive inhibitor
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
competitive inhibitor
O-(alpha-L-idopyranosyluronic acid)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
competitive inhibitor
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
-
When islets are cultured in different concentrations of D-glucose, the levels of enzyme mRNA increases, in a dose-dependent manner. Mannoheptulose completely blocks the effect of glucose.
-
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.0192
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol
-
-
0.0025 - 0.043
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
0.133 - 0.327
O-(alpha-L-idopyranosyluronic acid-2-sulfate)-(1-4)-2,5 anhydromannose-6-sulfate
0.0007 - 0.0011
O-(alpha-L-iduronic acid 2-sulfate)-(1-3)-2,5-anhydro-D-talitol 4-sulfate
-
pH dependent
0.0014
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
-
pH dependent
0.0019
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
-
pH dependent
0.0025
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
-
pH dependent
0.0031
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
-
pH dependent
0.0025 - 0.01
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
pH dependent
0.003
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
recombinant protein
0.0115
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
pool 1 from urine
0.012
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
in presence of 7 mM NaCl
0.013
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
pool 2 in urine
0.013
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
form A
0.015
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
form B
0.043
O-(alpha-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-mannitol 6-sulfate
-
in presence of 70 mM NaCl
0.133
O-(alpha-L-idopyranosyluronic acid-2-sulfate)-(1-4)-2,5 anhydromannose-6-sulfate
-
mutant R88H
0.327
O-(alpha-L-idopyranosyluronic acid-2-sulfate)-(1-4)-2,5 anhydromannose-6-sulfate
-
wild-type enzyme
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
0.000006 - 0.00066
-
hrIDS detection by ELISA-sandwich and fluorometric assay in Pichia pastoris culture samples, substrate is 4-methylumbelliferyl-2-iduronate-2-sulfate, pH not specified in the publication, temperature not specified in the publication, overview
0.00063 - 0.0021
-
enzyme determination in human plasma samples by a Fluorometric method, and ELISA double sandwich technique after several freeze and thawing cycles, substrate is 4-methylumbelliferyl-2-iduronate-2-sulfate, pH not specified in the publication, temperature not specified in the publication, overview
0.006
-
untreated neuronal cells, comparison to cells after adenoviral infection
0.00725
-
untreated control cells, comparison to cells after adenoviral infection
0.21
recombinant protein, pH not specified in the publication, temperature not specified in the publication
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
3.9 - 5.7
4.5 - 5.3
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
135568, 135570, 135571, 135573, 135574, 135575, 135576, 135577, 135578, 650331, 650341, 650371, 651461, 653245, 653249
-
-
brenda
-
707017, 707032, 707060, 707600, 708665, 709386, 709387, 709970, 709973, 710212, 714554, 715792, 730196, 730775
-
-
brenda
almost total absence of iduronate-2-sulfatase in patients with mucopolysaccharidosis type II compared with control
-
-
brenda
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
IDS activity already detected at the 64-cell stage, and increases at 6.5 hours post fertilization, during the onset of gastrulation. A gradual decrease of enzymatic activity from 24 hours post fertilization to 5 dpf
brenda
-
primary epithelial cell, highest activity of galacose 6-sulfatase and aryl sulfatase B of all the cell lines tested
brenda
-
IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation
brenda
-
IDS enzyme activity in mucopolysaccharidosis type II patient at 10 months post-cord blood stem cell transplantation
brenda
-
remarkably low activity of arylsulfatase A, arylsulfatase B, galacose 6-sulfatase and steryl sulfatase, but not iduronate 2-sulfatase
brenda
-
primary myoepithelial cell, highest activity of steryl sulfatase and aryl sulfatase B of all the cell lines tested
brenda
-
remarkably low activity of arylsulfatase A, arylsulfatase B, galacose 6-sulfatase and steryl sulfatase, but not iduronate 2-sulfatase
brenda
additional information
-
IDS is present in many cells of the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient as well as in the non-mucopolysaccharidosis type II control. Many IDS-immunoreactive large cells are found in the non-mucopolysaccharidosis type II control, whereas only a few IDS-immunoreactive small cells are found in the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient. Most IDS-positive small cells are localized in perivascular spaces in the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient. Many of them are in the distended Virchow-Robin spaces, and some of them in the brain parenchyma. No IDS-immunoreactive neurons in the mucopolysaccharidosis type II post-cord blood stem cell transplantation patient. IDS is present exclusively in CD68-positive microglia/monocytes in the brain of Hunter disease patient, whereas that in the normal brain is also detected in neurons and oligodendrocytes
brenda