GNS Rabbit Polyclonal Antibody
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Other products for "GNS"
Specifications
Product Data | |
Applications | WB |
Recommended Dilution | WB,1:500 - 1:2000 |
Reactivities | Human, Mouse, Rat |
Modifications | Unmodified |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | Recombinant fusion protein containing a sequence corresponding to amino acids 293-552 of human GNS (NP_002067.1). |
Formulation | Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |
Concentration | lot specific |
Purification | Affinity purification |
Conjugation | Unconjugated |
Stability | Shelf life: one year from despatch. |
Predicted Protein Size | 59kDa/62kDa |
Gene Name | glucosamine (N-acetyl)-6-sulfatase |
Database Link | |
Background | The product of this gene is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome. |
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