Iduronaat 2 sulfatase/IDS-eiwit, humaan, recombinant (hij)
Kwaliteit
ISO Gecertificeerd
Levering
24-48 uur
Technische Specificaties
0.966
Iduronate 2-Sulfatase, also known as IDS, is a member of the highly conserved sulfatase family of enzymes that catalyze the hydrolysis of O- and N-sulfate esters from a variety of substrates. The human Iduronate 2-Sulfatase/IDS consists of a signal peptide, a propeptide, and a mature chain that may be further processed into two chains. Among the identified 18 human sulfatases, Iduronate 2-Sulfatase/IDS is required for the lysosomal degradation of the glycosaminoglycans (GAG), heparan sulfate, and dermatan sulfate. Multiple mutations in this X-chromosome localized gene result in Iduronate 2-Sulfatase/IDS enzymatic deficiency and lead to the sex-linked Mucopolysaccharidosis Type II (MPS II), also known as Hunter Syndrome characterized by the lysosomal accumulation of the GAG and their excretion in urine. MPS II has a wide spectrum of clinical manifestations ranging from mild to severe due to the level of Iduronate 2-Sulfatase/IDS enzyme. Retroviral-mediated Iduronate 2-Sulfatase/IDS gene transfer into lymphoid cells would be a promising gene therapeutic strategy.
Ice Packs
-20°C
Beschrijving
Iduronaat 2 sulfatase/IDS-eiwit, humaan, recombinant (hij) Beschikbaar in 5 µg. Bestel eenvoudig online met snelle levering.
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