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Literature summary for 3.1.6.13 extracted from

  • Okuyama, T.; Tanaka, A.; Suzuki, Y.; Ida, H.; Tanaka, T.; Cox, G.; Eto, Y.; Orii, T.
    Japan Elaprase Treatment (JET) study: idursulfase enzyme replacement therapy in adult patients with attenuated Hunter syndrome (Mucopolysaccharidosis II, MPS II) (2010), Mol. Genet. Metab., 99, 18-25.
    View publication on PubMed

Application

Application Comment Organism
medicine idursulfase treatment appears to be safe and effective in adult Japanese patients with attenuated Mucopolysaccharidosis II Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Homo sapiens 5764
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Synonyms

Synonyms Comment Organism
iduronate-2-sulfatase
-
Homo sapiens
idursulfase
-
Homo sapiens

General Information

General Information Comment Organism
physiological function male patients, ages 21-53 years, receiving weekly intravenous infusions of 0.5 mg/kg idursulfase for 12 months, show significant reductions in lysosomal storage and several clinical improvements. Idursulfase is generally well-tolerated. Infusion-related reactions occur in 50% of patients and are mostly mild with transient skin reactions that do not require medical intervention. Two infusion-related reactions are assessed as serious (urticaria and vasovagal syncope) Homo sapiens