GNS Rabbit Polyclonal Antibody
Product Data | |
Application | WB |
---|---|
Recommended Dilution | Western blot: 1:50 - 1:100. ELISA: 1:1,000. |
Reactivity | Human |
Antibody Host | Rabbit |
Clonality | Polyclonal |
Immunogen | KLH conjugated synthetic peptide selected from the Center region of human GNS |
Specificity | This antibody detects GNS at Center, N189. |
Buffer | PBS with 0.09% (W/V) sodium azide State: Liquid Ig fraction |
Concentration | lot specific |
Purification | Prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS |
Conjugation | Unconjugated |
Storage | Store the antibody at 2 - 8 °C up to one month or (in aliquots) at -20 °C for longer. Avoid repeated freezing and thawing. |
Stability | Shelf life: one year from despatch. |
Shipping | Blue Ice |
Gene Name | glucosamine (N-acetyl)-6-sulfatase |
Database Link | |
Background | GNS 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. |
Synonyms | Glucosamine-6-sulfatase |
Note | Molecular weight: 62082 Da |
Reference Data | |
Protein Families | Druggable Genome, Transmembrane |
Protein Pathways | Glycosaminoglycan degradation, Lysosome, Metabolic pathways |
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