| Attributes | Values |
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| rdf:type
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| http://linked.open...gbank/description
| - Idursulfase is a purified form of human iduronate-2-sulfatase, a lysosomal enzyme. Idursulfase is produced by recombinant DNA technology in a human cell line. Idursulfase is an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the glycosaminoglycans dermatan sulfate and heparan sulfate in the lysosomes of various cell types. Idursulfase is a 525-amino acid glycoprotein with a molecular weight of approximately 76 kilodaltons. The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide structures. The enzyme activity of idursulfase is dependent on the post-translational modification of a specific cysteine to formylglycine. (en)
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| http://linked.open...y/drugbank/dosage
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| http://linked.open...generalReferences
| - # Garcia AR, DaCosta JM, Pan J, Muenzer J, Lamsa JC: Preclinical dose ranging studies for enzyme replacement therapy with idursulfase in a knock-out mouse model of MPS II. Mol Genet Metab. 2007 Jun;91(2):183-90. Epub 2007 Apr 24. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/17459751 # Zareba G: Idursulfase in Hunter syndrome treatment. Drugs Today (Barc). 2007 Nov;43(11):759-67. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/18174963 # Clarke LA: Idursulfase for the treatment of mucopolysaccharidosis II. Expert Opin Pharmacother. 2008 Feb;9(2):311-7. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/18201153 # Burrow TA, Leslie ND: Review of the use of idursulfase in the treatment of mucopolysaccharidosis II. Biologics. 2008 Jun;2(2):311-20. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/19707363 # Martin RA: Mucopolysaccharidosis Type II "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/20301451 # Wraith JE, Scarpa M, Beck M, Bodamer OA, De Meirleir L, Guffon N, Meldgaard Lund A, Malm G, Van der Ploeg AT, Zeman J: Mucopolysaccharidosis type II (Hunter syndrome): a clinical review and recommendations for treatment in the era of enzyme replacement therapy. Eur J Pediatr. 2008 Mar;167(3):267-77. Epub 2007 Nov 23. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/18038146 (en)
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| http://linked.open...gy/drugbank/group
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| http://linked.open...drugbank/halfLife
| - 44 ± 19 minutes (en)
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| http://linked.open...ugbank/indication
| - For the treatment of Hunter syndrome in adults and children ages 5 and older. (en)
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| sameAs
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| Title
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| adms:identifier
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| http://linked.open...mechanismOfAction
| - Hunter's Syndrome is an X-linked recessive disease caused by insufficient levels of the lysosomal enzyme iduronate-2-sulfatase. This enzyme cleaves the terminal 2-O-sulfate moieties from the glycosaminoglycans (GAG) dermatan sulfate and heparan sulfate. Due to the missing or defective iduronate-2-sulfatase enzyme in patients with Hunter's Syndrome, GAG progressively accumulate in the lysosomes of a variety of cells, leading to cellular engorgement, organomegaly, tissue destruction and organ system dysfunction. Treatment of Hunter's Syndrome patients with idursulfase provides exogenous enzyme for uptake into cellular lysosomes. Targeting of idursulfase to the lysosome occurs by endocytosis from the cell surface. Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow specific binding of the enzymes to the M6P receptors on the cell surface, leading to cellular internalization of the enzyme, targeting to intracellular lysosomes and subsequent catabolism of accumulated GAG. (en)
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| http://linked.open...drugbank/packager
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| http://linked.open.../drugbank/synonym
| - Alpha-L-iduronate sulfate sulfatase (en)
- Iduronate 2-sulfatase (en)
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| http://linked.open...drugbank/toxicity
| - There is no experience with overdosage of Idursulfase in humans. Single intravenous doses of idursulfase up to 20 mg/kg were not lethal in male rats and cynomolgus monkeys (approximately 6.5 and 13 times, respectively, of the recommended human dose based on body surface area) and there were no clinical signs of toxicity. (en)
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| foaf:page
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| http://linked.open...Molecular-Formula
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| http://linked.open.../Molecular-Weight
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| http://linked.open...l/drug/hasATCCode
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| http://linked.open.../affectedOrganism
| - Humans and other mammals (en)
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| http://linked.open...casRegistryNumber
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| http://linked.open...drugbank/category
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| http://linked.open...rugbank/clearance
| - * 3 mL/min/kg [Patients (7.7 – 27 years) with Hunter syndrome with treatment week 1(0.5 mg/kg ELAPRASE administered weekly as a 3-hour infusion)] * 3.4 mL/min/kg [patients (7.7 – 27 years) with Hunter syndrome with treatment week 27 (0.5 mg/kg ELAPRASE administered weekly as a 3-hour infusion)] (en)
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| http://linked.open...gbank/containedIn
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