The GNS Antibody (CAB7489) is a high-quality antibody developed for reliable detection and analysis of target proteins. 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).
This antibody is validated for use in WB, ELISA applications and has demonstrated reactivity against Human, Mouse, Rat samples.
Product Name:
GNS Antibody
SKU:
CAB7489
Size:
20μL, 100μL
Reactivity:
Human, Mouse, Rat
Conjugate:
Unconjugated
Immunogen:
Recombinant protein (or fragment).This information is considered to be commercially sensitive.
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
RRID
AB_2768020
Buffer Information
Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS containing 50% glycerol, preserved with proclin300 or sodium azide, pH 7.3.
Western blot analysis of various lysates using GNS Rabbit pAb (CAB7489) at 1:1000 dilution. Secondary antibody: HRP-conjugated 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.
