3.2.1.76: L-iduronidase
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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
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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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Application on EC 3.2.1.76 - L-iduronidase
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APPLICATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
diagnostics
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determining residual IDUA activity in fibroblasts of MPS I patients may be helpful to predict MPS I phenotype. Early recognition of the phenotype of MPS I patients is essential to timely initiate the most appropriate therapeutic strategy
drug development
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mutant AGT-181 might be useful as a therapeutic approach to treatment of the brain in Hurlers syndrome
medicine
pharmacology
medicine
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the enzyme is a target for enzyme replacement therapy of lysosomal storage disorders of mucopolysaccharidosis type I patients, the therapy depends on efficient uptake of recombinant enzyme into tissues of patients
medicine
the enzyme is a target for enzyme replacement therapy of mucopolysaccharidosis type I patients
medicine
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the recombinant enzyme is used in enzyme relacement therapy of the Hurler form of mucopolysaccharidosis type I, MPSI, the enzyme is administered intraventricularly in to 31 Sprague-Dawley rat brains showing penetration of the brain and uptak into neurons and glial cells, rat tissue distribution of human recombinant enzyme activity, overview
medicine
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administration of a high dose of the enzyme or development of a recombinant alpha-L-iduronidase containing many mannose 6-phosphate residues is required for further improvement of enzyme replacement therapy for skeletal disorders caused by mucopolysaccharidosis I
medicine
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as there is a difference in IDUA structural change between the severe mucopolysaccharidosis type I group and the attenuated one, except for a couple of mutations, structural analysis can help predict the clinical outcome of the disease
medicine
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commercially available recombinant human laronidase (Aldurazyme) infusion is safe and effective in stabilizing or improving pulmonary function and physical endurance. Preclinical trials of the enzyme in the canine, dog and feline model and clinicial trials with affected patients with mucopolysaccharidosis type I
medicine
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currently approved laronidase dose regimen, to treat the lysosomal storage disorder mucopolysaccharidosis type I, has similar efficacy and potentially improved safety compared to regimens using higher doses, regardless of dose frequency. The approved 0.58 mg/kg/week laronidase dose regimen provides near-maximal reductions in glycosaminoglycan storage and the best benefit-to-risk ratio. The 1.2 mg/kg every 2 weeks regimen may be an acceptable alternative for patients with difficulty receiving weekly infusions, but the long-term effects of this regimen are unknown. In general, laronidase therapy is safe and well tolerated in all treatment groups
medicine
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long-term clinical benefit and safety of laronidase in attenuated patients with mucopolysaccharidosis I. Laronidase effectively treats respiratory dysfunction, poor endurance, restricted mobility, hepatomegaly, and decreased quality of life. Prompt disease recognition and early and sustained treatment with laronidase will maximize these important benefits of treatment
medicine
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the benefit of enzyme replacement therapy with recombinant laronidase before hematopoietic stem cell transplantation for mucopolysaccharidosis I is linked to improvement in patient's pretransplantation condition and thus tolerance of such intensive therapy. Short-term use of laronidase is not associated with increased risk of either graft-versus-host-disease or graft failure
medicine
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effectiveness of enzyme replacement therapy with laronidase on the range of motion of upper extremities and influence on activities of daily living of patients with mucopolysaccharidosis type I, MPS I, overview. MPS I has a spectrum of clinical severity, and is subdivided into three phenotypes: Hurler syndrome, that is severe, Hurler-Scheie syndrome, that is intermediate, and Scheie syndrome, that is attenuated
medicine
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the enzyme is useful in treatment of mucopolysaccharidosis type I, MPS I. In three patients with attenuated MPSA I, treated by laronidase, patients 2 and 3 display significant cognitive improvement within 2 years, and patients 1 and 3 display improvement, on MRI scans of the brain, overview
medicine
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the enzyme is useful in treatment of mucopolysaccharidosis type I, MPS I, by enzyme replacement therapy
medicine
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intrathecal administration of recombinant human IDU, with potential dosing every 2-3 months, may provide benefit for the treatment of central nervous system disease in mucopolysaccharidosis type I patients
medicine
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intrathecal administration of recombinant human IDU, with potential dosing every 2-3 months, may provide benefit for the treatment of central nervous system disease in mucopolysaccharidosis type I patients
medicine
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a clone overexpressing the enzyme and, after encapsulation in alginate microcapsules, correcting MPS I human skin fibroblasts.These capsules can be surgically implanted in sites which are difficult to reach such as the brain of animal models and can be an approach for the treatment of MPS I and other lysosomal storage disorders
medicine
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alpha-L-iduronidase is used in enzyme replacement therapy approved for mucopolysaccharidosis type I treatment
pharmacology
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exploitation of alternative receptor systems that are independent of glycosylation but allow for efficient delivery to the lysosome
pharmacology
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the enzyme is used in enzyme replacement therapy of mucopolysaccharidosis type I, MPSI, changes in hair morphology of MPSI patients treated with recombinant human enzyme, overview
