GNS Rabbit Polyclonal Antibody
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Other products for "GNS"
Specifications
Product Data | |
Applications | IHC, WB |
Recommended Dilution | WB: 500-2000 WB positive control: PC3 cell lysate IHC: 50-200 Positive control: Human cervical cancer Predicted cell location: Cytoplasm |
Reactivities | Human, Mouse |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | Fusion protein of human GNS |
Formulation | pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
Concentration | lot specific |
Purification | Antigen affinity purification |
Conjugation | Unconjugated |
Storage | Store at -20°C. |
Stability | 1 year |
Predicted Protein Size | 62 kDa |
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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