Anti-Iduronaat 2 sulfatase/IDS Antibody Picoband® 0,00647 Vervoeging

IDS (Iduronate-2-sulfatase) is a sulfatase enzyme associated with Hunter syndrome. Iduronate 2-sulfatase is involved in the lysosomal degradation of the glycosaminoglycans heparan sulfate and dermatan sulfate. Wilson et al. (1991) used an IDS cDNA clone to localize the gene to Xq28, distal to the fragile X site. Faust et al. (1992) and Daniele et al. (1993) demonstrated that the homologous Ids gene in the mouse occupies the same position on the X chromosome in relation to the FMR1, F9, and GABRA3 genes. 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.

Iduronate 2-sulfatase;3.1.6.13; Alpha-L-iduronate sulfate sulfatase; Idursulfase; Iduronate 2-sulfatase 42 kDa chain; Iduronate 2-sulfatase 14 kDa chain; IDS; SIDS

IDS

3423

P22304

Human

No cross-reactivity with other proteins

A synthetic peptide corresponding to a sequence at the C-terminus of human Iduronate 2 sulfatase.

Liver, kidney, lung, and placenta.

Flow Cytometry

Growth Factors/Hormones, Hematopoietic Progenitors, Lymphoid, Mesenchymal Stem Cells, Signal Transduction, Stem Cells

Immunogen affinity purified.

Liquid

Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.

1. Daniele, A., Faust, C. J., Herman, G. E., Di Natale, P., Ballabio, A. Cloning and characterization of the cDNA for the murine iduronate sulfatase gene. Genomics 16: 755-757, 1993. 2. Faust, C. J., Verkerk, A. J. M. H., Wilson, P. J., Morris, C. P., Hopwood, J. J., Oostra, B. A., Herman, G. E. Genetic mapping on the mouse X chromosome of human cDNA clones for the fragile X and Hunter syndromes. Genomics 12: 814-817, 1992. 3. Wilson, P. J., Suthers, G. K., Callen, D. F., Baker, E., Nelson, P. V., Cooper, A., Wraith, J. E., Sutherland, G. R., Morris, C. P., Hopwood, J. J. Frequent deletions at Xq28 indicate genetic heterogeneity in Hunter syndrome. Hum. Genet. 86: 505-508, 1991.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

61873 MW

No cross reactivity with other proteins.

6

Iduronate 2-sulfatase

Lysosome.

Contains 2 follistatin-like domains.

Iduronate 2-sulfatase

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.