|Catalog||Size (Price)||Quantity||In Stock||Operation||Other Information|
GNS / G6S Antibody
|Order or Inquire for GNS Antibody product||Quality antibodies||Antibody production services|
|Detection limit is 0.125 ng/lane in WB|
|Detection limit is 0.00245 ng/well in ELISA|
GNS / G6S Antibody Product Information
|Antibody Type :||Rabbit Monoclonal Antibody ( Rabbit mAb Service Platform )|
Clone ID :
|Ig Type :||
|Formulation :||0.2 μm filtered solution in PBS with 5% trehalose|
GNS / G6S Antibody Usage Guide
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
|Molecule||Species||Description //For Detailed Info. and Price------CLICK!||Cat. No|
|GNS/G6S||Human||GNS/G6S Protein, Recombinant||10319-H08H|
|Molecule||Application||Description //For Detailed Info. and Price------CLICK!||Cat. No|
|WB, ELISA||Mouse Monoclonal Antibody||10319-MM01|
|WB, ELISA||GNS/G6S Antibody, Rabbit MAb||10319-R007|
|WB, ELISA||Rabbit Polyclonal Antibody||10319-RP02|
|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.
- Tomatsu, S. et al., 1991, Biochem. Biophys. Res. Commun. 181: 677-83.
- Mok, A. et al., 2003, Genomics 81: 1-5.
- Parenti, G. et al., 1997, Curr. Opin. Genet. & Dev. 7: 386-91.
- Beesley, CE. et al., 2003, J. Med. Genet. 40:192-4
Glucosamine (N-acetyl)-6-Sulfatase related areas, pathways, and other information