3.2.1.76: L-iduronidase
This is an abbreviated version!
For detailed information about L-iduronidase, go to the full flat file.
Word Map on EC 3.2.1.76
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3.2.1.76
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mucopolysaccharidosis
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lysosomal
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hurler
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glycosaminoglycans
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scheie
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dermatan
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heparan
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hurler-scheie
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hematopoietic
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corneal
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medicine
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mucopolysaccharide
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intrathecal
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clouding
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hepatosplenomegaly
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iduronate
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pompe
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pharmacology
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enzyme-replacement
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4-methylumbelliferyl
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facies
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mckusick
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diagnostics
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dysostosis
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fabry
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drug development
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gaucher
- 3.2.1.76
- mucopolysaccharidosis
- lysosomal
- hurler
- glycosaminoglycans
-
scheie
- dermatan
- heparan
-
hurler-scheie
-
hematopoietic
- corneal
- medicine
-
mucopolysaccharide
-
intrathecal
-
clouding
-
hepatosplenomegaly
-
iduronate
- pompe
- pharmacology
-
enzyme-replacement
-
4-methylumbelliferyl
-
facies
-
mckusick
- diagnostics
-
dysostosis
- fabry
- drug development
- gaucher
Reaction
Synonyms
Aldurazyme, alpha-IDUA, alpha-iduronidase, alpha-L-iduronidase, alpha-L-iduronidase A, glycosaminoglycan alpha-L-iduronohydrolase, hIDUA, IDU, IDUA, iduronidase, laronidase, More, rIDUA_RLT
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3.2.1.76 L-iduronidaseAdvanced search results
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Natural Substrates Products on EC 3.2.1.76 - L-iduronidase
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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
4-methylumbelliferyl alpha-L-iduronide + H2O
4-methylumbelliferone + alpha-L-iduronic acid
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alpha-L-iduronosyl-(1->3)-2,5-anhydro-D-talitol 4-sulfate + H2O
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additional information
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cleaves alpha-L-iduronic acid from the nonreducing ends of dermatan sulfate, heparin, and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I
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additional information
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involved in the degradation of dermatan sulfate and heparan sulfate
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additional information
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involved in the degradation of dermatan sulfate and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I
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additional information
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involved in the degradation of dermatan sulfate and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I
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additional information
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involved in the degradation of dermatan sulfate and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I
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additional information
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involved in the degradation of dermatan sulfate and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I
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additional information
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enzyme deficiency causes mucopolysaccharidosis type I, MPSI
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additional information
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enzyme deficiency causes the lysosomal storage disorder mucopolysaccharidosis type I, MPSI
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additional information
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enzyme deficiency causes the lysosomal storage disorder mucopolysaccharidosis type I, MPSI
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additional information
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enzyme deficiency causes the lysosomal storage disorder mucopolysaccharidosis type I, MPSI
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additional information
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the enzyme is involved in mucopolysaccharidosis type I
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additional information
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the enzyme is involved in mucopolysaccharidosis type I
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additional information
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the enzyme is involved in the sequential degradation of heparan sufate and dermatan sulfate, enzyme deficiency results in the accumulation and urinary secretion of partially degraded glycosaminoglycans and causes the lysosomal storage disorder mucopolysaccharidosis type I, MPSI
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additional information
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the enzyme cleaves terminal alpha-iduronic acid residues from glycosaminoglycan, heparan sulfate and dermatan sulfate
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additional information
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the enzyme hydrolyses the glycosidic bond between the terminal L-iduronic acid and the second sugar of N-acetylgalactosamine-4-sulfate/N-sulfo-D-glucosamine-6-sulfate, which are the major components of dermatan/heparan sulfate
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additional information
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the enzyme hydrolyzes terminal iduronic acid residues on glycosaminoglycans
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