Human Iduronate 2-Sulfatase/IDS Antibody Summary
Ser26-Pro550
Accession # P22304
Applications
Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.
Reconstitution Calculator
Preparation and Storage
- 12 months from date of receipt, -20 to -70 °C as supplied.
- 1 month, 2 to 8 °C under sterile conditions after reconstitution.
- 6 months, -20 to -70 °C under sterile conditions after reconstitution.
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). Recombinant human IDS corresponds to the single chain and has sulfatase activity described above.
- 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 - 3452, New York, McGraw-Hill.
Product Datasheets
Citation for Human Iduronate 2-Sulfatase/IDS Antibody
R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions.
1 Citation: Showing 1 - 1
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In vivo genome editing of the albumin locus as a platform for protein replacement therapy
Authors: Rajiv Sharma, Xavier M. Anguela, Yannick Doyon, Thomas Wechsler, Russell C. DeKelver, Scott Sproul et al.
Blood
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