Anti-GNS Antibody

Cat: 10319-MM01
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Anti-GNS Antibody (Mouse Monoclonal antibody) General Information
Product name
Anti-GNS Antibody
Validated applications
ELISA
Species reactivity
Reacts with: Human
Specificity
Human GNS
Immunogen
Human cell-derived rhGNS (Catalog#10319-H08H)
Preparation
This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human GNS / G6S (rhGNS; Catalog#10319-H08H; NP_002067.1; Met 1-Leu 552). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
Source
Monoclonal Mouse IgG2b Clone #7D5H8H11
Purification
Protein A
Formulation
0.2 μm filtered solution in PBS with 5% trehalose
Conjugate
Unconjugated
Form
Liquid
Shipping
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
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 -80℃. 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.
Anti-GNS Antibody (Mouse Monoclonal antibody) Validated Applications
Application Dilution Notes
ELISA 1:1000-1:2000  

**********Please Note: Optimal concentrations/dilutions should be determined by the end user.**********

Anti-GNS Antibody Alternative Names
Anti-G6S Antibody
GNS Background Information

Glucosamine (N-acetyl)-6-sulfatase (GNS), also known as G6S, a hydrolase, which is one of the enzymes involved in heparan sulfate catabolism leading to lysosomal storage. 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. Mucopolysaccharidosis type IIID (MPS IIID) is the least common of the four subtypes of Sanfilippo syndrome. It is caused by a deficiency of N-acetylglucosamine-6-sulphatase. A mutation in GNS resulting in MPS IIID indicates the potential utility of molecular diagnosis for this rare condition. As the least common type of the four subtypes of Sanfilippo syndrome, MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations.

Full Name
glucosamine (N-acetyl)-6-sulfatase
References
  • Fuchs W, et al. (1985) Intralysosomal formation and metabolic fate of N-acetylglucosamine 6-sulfate from keratan sulfate. Eur J Biochem. 151(3): 551-6.
  • Beesley CE, et al. (2003) Sanfilippo syndrome type D: identification of the first mutation in the N-acetylglucosamine-6-sulphatase gene. J Med Genet. 40(3): 192-4.
  • Mok A, et al. (2003) Genomic basis of mucopolysaccharidosis type IIID (MIM 252940) revealed by sequencing of GNS encoding N-acetylglucosamine-6-sulfatase. Genomics. 81(1): 1-5.
  • Elioglu NH, et al. (2009) A novel loss-of-function mutation in the GNS gene causes Sanfilippo syndrome type D. Genet Couns. 20(2): 133-9.
  • Product Description Host Clonality Application Catalog# (PDF)
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