363552
IDS (Iduronate-2-Sulfatase, MPS2, SIDS, Hunter Syndrome, Idursulfase, Alpha-L-iduronate sulfate sulfatase)
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£577.00
- SKU:
- 363552
- Reinheit:
- Purified by Protein A and peptide affinity chromatography.
- Lagerbedingungen:
- -20°C
- Hersteller:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Recombinant protein corresponding to Ile107-Thr363 of rat IDS, expressed in E. coli.
- Format:
- Supplied as a liquid in 0.01M PBS, pH7.4, 0.05% Proclin-300, 50% glycerol.
- Weitere Details:
- Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-chromosome gene that result in enzymatic deficiency lead to the sex-linked Mucopolysaccharidosis Type II, also known as Hunter Syndrome. Iduronate-2-sulfatase has a strong sequence homology with human arylsulfatases A, B, and C, and human glucosamine-6-sulfatase. A splice variant of this gene has been described. Two forms of MPS II are distinguishable clinically. A severe form (called MPS IIA in my nomenclature) has progressive mental retardation and physical disability and death before age 15 years in most cases. A mild form (called MPS IIB) is compatible with survival to adulthood, and reproduction is known to have occurred. Applications: Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Versandbedingungen:
- Blue Ice