The GNS Polyclonal Antibody (CAB7489) is a valuable tool for researchers studying GNS, a glycoside hydrolase enzyme involved in the breakdown of glycosphingolipids. This antibody, produced in rabbits, is highly specific for human samples and has been validated for use in Western blot applications. By binding to the GNS protein, this antibody enables the detection and analysis of GNS in a variety of cell types, making it an essential reagent for studies in lysosomal storage disorders, lipid metabolism, and related diseases.GNS plays a crucial role in the degradation of glycosphingolipids within lysosomes, and mutations in the GNS gene can lead to severe lysosomal storage disorders such as mucopolysaccharidosis type IIID (Sanfilippo syndrome type D).
Research on GNS is important for understanding the mechanisms of lysosomal function and lipid metabolism, as well as for developing potential therapies for these devastating genetic disorders. The GNS Polyclonal Antibody (CAB7489) is a valuable resource for scientists investigating these critical areas of biomedical research.
Product Name:
GNS Rabbit Polyclonal Antibody
SKU:
CAB7489
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse,Rat
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 293-552 of human GNS (NP_002067.1).
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.
Purification Method:
Affinity purification
Gene ID:
2799
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Western blot analysis of extracts of various cell lines, using GNS antibody (CAB7489) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit (AbGn00020).Exposure time: 30s.
