Iduronate 2-Sulfatase/IDS Antibody [Alexa Fluor™ Plus 647]

• Reactivity: Hu
• Clonality: Polyclonal
• Host: Goat
• Conjugate: Alexa Fluor Plus 647

Iduronate 2-Sulfatase/IDS Antibody [Alexa Fluor™ Plus 647] Summary

• Specificity: Detects human IDS in direct ELISAs and Western blots. In direct ELISAs, approximately 50% cross-reactivity with recombinant mouse IDS is observed.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Goat

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for Iduronate 2-Sulfatase/IDS Antibody [Alexa Fluor™ Plus 647]

• Alpha-L-iduronate sulfate sulfatase
• EC 3.1.6.13
• IDS
• iduronate 2-sulfatase 14 kDa chain
• iduronate 2-sulfatase 42 kDa chain
• iduronate 2-sulfatase
• idursulfase
• MPS2
• S
• SIDS

Background

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.

1. Wilson, P.J. et al. (1990) Proc. Natl. Acad. Sci. USA 87:8531.
2. Parenti, G. et al. (1997) Curr. Opin. Genet. & Dev. 7:386.
3. Neufeld, E.F. and J. Muenzer (2001) in The Metabolic and Molecular Basis of Inherited Disease, Scriver, C.R. et al. (eds.) pp. 3421-3452, New York, McGraw-Hill.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.