GNS / G6S Antibody

GNS / G6S Antibody

GNS / G6S Antibody Product Information

• Immunogen: Recombinant human GNS protein ( Catalog#10319-H08H )
• Antibody Type: Rabbit Monoclonal Antibody ( Rabbit mAb Service Platform )
• Clone ID: 007
• Ig Type: Rabbit IgG
• Formulation: 0.2 μm filtered solution in PBS with 5% trehalose
• Preparation: This antibody was obtained from a rabbit immunized with purified, human cell-derived, recombinant human GNS / G6S ( rhGNS ; Catalog#10319-H08H ; NP_002067.1 ; Met 1 - Leu 552 ).

GNS / G6S Antibody Usage Guide

• Specificity: Human GNS / G6S
• Western blot: This antibody can be used at 1 - 2 μg/mL with the appropriate secondary reagents to detect human GNS in WB. Using a DAB detection system, the detection limit for human GNS is approximately 0.125 ng/lane under non-reducing conditions and 0.5 ng/lane under reducing conditions
• Direct ELISA: This antibody can be used at 0.1 - 0.2 μg/mL with the appropriate secondary reagents to detect human GNS. The detection limit for human GNS is approximately 0.00245 ng/well
• Storage: This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -70℃. Preservative-Free.
  Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles.

GNS / G6S Antibody Related Products & Topics

Related Areas:

Enzyme>>Carbohydrate Metabolism Enzymes>>Glucosamine (N-acetyl)-6-Sulfatase/GNS

Immunology>>Innate Immunity>>Lysosomal Enzyme>>Glucosamine (N-acetyl)-6-Sulfatase/GNS

Proteins:

Molecule	Species	Description //For Detailed Info. and Price------CLICK!	Cat. No
GNS/G6S	Human	GNS/G6S Protein, Recombinant	10319-H08H

Antibodies:

Molecule	Application	Description //For Detailed Info. and Price------CLICK!	Cat. No
Human GNS/G6S	WB, ELISA	Mouse Monoclonal Antibody	10319-MM01
Human GNS/G6S	WB, ELISA	GNS/G6S Antibody, Rabbit MAb	10319-R007
Human GNS/G6S	WB, ELISA	Rabbit Polyclonal Antibody	10319-RP02
Human GNS/G6S	WB, ELISA	Rabbit Polyclonal Antibody (Antigen Affinity Purified)	10319-RP03

GNS / G6S Antibody Background

Human glucosamine (N-acetyl)-6-sulfatase ( GNS ), also known as G6S, is a lysosomal enzyme ubiquitously present in cells. GNS is required for the catabolism of the glycosaminoglycans (GAG) including heparin, heparan sulphate, and keratan sulphate through the hydrolysis of 6-sulfate group from the N-acetyl-D-glucosamine 6-sulfate units. The deficiency or loss-of-function of GNS result in mucopolysaccharidosis type IIID (MPS IIID), an inborn catabolism disorder with the accumulation of undegraded substrate and the lysosomal storage. As the least common type of the four subtypes of Sanfilippo syndrome, MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations. 

References

1. Tomatsu, S. et al., 1991, Biochem. Biophys. Res. Commun. 181: 677-83.
2. Mok, A. et al., 2003, Genomics 81: 1-5.
3. Parenti, G. et al., 1997, Curr. Opin. Genet. & Dev. 7: 386-91.
4. Beesley, CE. et al., 2003, J. Med. Genet. 40:192-4