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Idursulfase

Idursulfase Struktur
50936-59-9
CAS-Nr.
50936-59-9
Englisch Name:
Idursulfase
Synonyma:
Idursulfase;L-Iduronate 2-sulfate sulfatase
CBNumber:
CB42502845
Summenformel:
Molgewicht:
0
MOL-Datei:
Mol file

Idursulfase Eigenschaften

Sicherheit

Idursulfase Chemische Eigenschaften,Einsatz,Produktion Methoden

Beschreibung

Mucopolysaccharidosis II, also known as Hunter Syndrome, is a lysosomal storage disorder characterized by a deficiency in iduronate-2-sulfatase, an enzyme responsible for the hydrolysis of the terminal 2-sulfate esters from the glycosaminoglycans dermatan sulfate and heparin sulfate in the lysosomes of various cells. This enzyme deficiency causes an accumulation of glycosaminoglycans (GAGs) in tissue. The clinical manifestations of this deficiency are short stature, joint stiffness, harsh facial features, hepatosplenomegaly, and progressive mental retardation. As with other lysosomal storage disorders, the patient’s only recourse is enzyme replacement therapy (ERT). Idursulfase is a recombinant human enzyme that has been developed and launched as the ERT for Hunter syndrome. Unlike most recombinant enzymes, it cannot be produced in prokaryotic cells. For proper post-translational attachment of N-linked oligosaccharides and the crucial mannose-6-phosphate groups as the targeting passport into lysosomes, idursulfase is produced from HT-1080 cells. In addition to being fully glycosylated with eight mannose-6-phosphate groups, the enzyme possesses sialylated moieties that improve its stability in circulation.

Verwenden

Treatment of Hunter Syndrome (enzyme that degrades the glycosaminoglycans heparan sulfate and dermatan sulfate).

Idursulfase Upstream-Materialien And Downstream Produkte

Upstream-Materialien

Downstream Produkte


Idursulfase Anbieter Lieferant Produzent Hersteller Vertrieb H?ndler.

Global( 5)Lieferanten
Firmenname Telefon E-Mail Land Produktkatalog Edge Rate
Creative Enzymes
1-516-855-7709
info@creative-enzymes.com United States 8748 58
LGM Pharma 1-(800)-881-8210
inquiries@lgmpharma.com United States 2123 70
Beijing HuaMeiHuLiBiological Chemical 010-56205725
waley188@sohu.com China 12335 58
Chizhou Kailong Import and Export Trade Co., Ltd.
xg01_gj@163.com China 9484 50

  • Idursulfase
  • L-Iduronate 2-sulfate sulfatase
  • 50936-59-9
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