Recombinant Human Iduronate 2-Sulfatase Protein, CF
R&D Systems, part of Bio-Techne | Catalog # 2449-SU
Key Product Details
Product Specifications
Source
Ser26-Pro550, with a C-terminal 10-His tag
Purity
Endotoxin Level
N-terminal Sequence Analysis
Predicted Molecular Mass
SDS-PAGE
Activity
The specific activity is > 1.0 pmol/min/µg, as measured under the described conditions.
Formulation, Preparation and Storage
2449-SU
Formulation | Supplied as a 0.2 μm filtered solution in Tris and NaCl. |
Shipping | The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below. |
Stability & Storage | Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
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Background: Iduronate 2-Sulfatase/IDS
As a member of the sulfatase family, IDS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and dermatan sulfate (2, 3). It hydrolyzes the 2-sulfate group of the L-iduronate 2-sulfate units of the GAG. The IDS deficiency results in mucopolysaccharidosis II (MPS II or Hunter syndrome), an X-linked inborn error leading to lysosomal accumulation of the GAG and its excretion in urine. MPS II has a wide spectrum of clinical manifestations ranging from mild to severe. The deduced amino acid sequence of human IDS consists of a signal peptide (residues 1‑25), a pro peptide (residues 26‑33) and a mature chain (residues 34‑550) that may be further processed into the 42 kDa chain (residues 34‑455) and the 14 kDa chain (residues 456‑550) (1). rhIDS corresponds to the single chain and has sulfatase activity described above.
References
- Wilson, P.J. et al. (1990) Proc. Natl. Acad. Sci. USA 87:8531.
- Parenti, G. et al. (1997) Curr. Opin. Genet. & Dev. 7:386.
- Neufeld, E.F. and Muenzer, J. (2001) in The Metabolic and Molecular Basis of Inherited Disease, Scriver, C.R. et al. (eds.) pp. 3421, New York, McGraw-Hill.
Alternate Names
Gene Symbol
UniProt
Additional Iduronate 2-Sulfatase/IDS Products
Product Documents for Recombinant Human Iduronate 2-Sulfatase Protein, CF
Product Specific Notices for Recombinant Human Iduronate 2-Sulfatase Protein, CF
For research use only