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EC Number Application Commentary Reference
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76diagnostics 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 732511
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76drug development mutant AGT-181 might be useful as a therapeutic approach to treatment of the brain in Hurler’s syndrome 709198
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine 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 731638
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine 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 696554
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine alpha-L-iduronidase is used in enzyme replacement therapy approved for mucopolysaccharidosis type I treatment 732391
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine 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 699314
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine 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 697827
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine 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 700229
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine 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 709388
Display the word mapDisplay the reaction diagram Show all sequences 3.2.1.76medicine hIDUA is used for enzyme replacement therapy in MPS I patients 710324
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