Human Glucosamine (N-acetyl)-6‑Sulfatase/GNS Alexa Fluor™ Plus 405-conjugated Antibody
R&D Systems, part of Bio-Techne | Catalog # AF2484AFP405
Conjugate
Catalog #
Key Product Details
Species Reactivity
Human
Applications
Western Blot, Immunoprecipitation
Label
Alexa Fluor Plus 405 (Excitation = 404 nm, Emission = 455 nm)
Antibody Source
Polyclonal Goat IgG
Product Specifications
Immunogen
Mouse myeloma cell line NS0-derived recombinant human Glucosamine (N-acetyl)‑6-Sulfatase/GNS
Specificity
Detects human Glucosamine (N-acetyl)-6‑Sulfatase/GNS in direct ELISAs and Western blots.
Clonality
Polyclonal
Host
Goat
Isotype
IgG
Applications
Application
Recommended Usage
Immunoprecipitation
Optimal dilution of this antibody should be experimentally determined.
Western Blot
Optimal dilution of this antibody should be experimentally determined.
Formulation, Preparation, and Storage
Formulation
Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.
Shipping
The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage
Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
Background: Glucosamine (N-acetyl)-6-Sulfatase/GNS
A member of the sulfatase family, GNS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and keratan sulfate (1, 2). It hydrolyzes the 6-sulfate group of the N-acetyl-D-glucosamine 6-sulfate units of the GAG. GNS deficiency results in mucopolysaccharidosis type IIID (MPS IIID or Sanfilippo D Syndrome), an inborn error leading to lysosomal accumulation of heparan sulfate. MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations. The deduced amino acid sequence of human GNS consists of a signal peptide (residues 1‑36) and a mature chain (residues 37‑552) that may be further processed into N-terminal and C-terminal fragments (3). Recombinant human GNS corresponds to the single chain and has sulfatase activity.
References
- Parenti, G. et al. (1997) Curr. Opin. Genet. & Dev. 7:386.
- Neufeld, E.F. and J. Muenzer (2001) in The Metabolic and Molecular Basis of Inherited Disease, Scriver, C.R. et al. (eds.) pp. 3421 - 3452, New
- Robertson, D.A. et al. (1992) Biochem. J. 288:539.
Alternate Names
Glucosamine6Sulfatase, GNS
Entrez Gene IDs
2799 (Human)
Gene Symbol
GNS
UniProt
Additional Glucosamine (N-acetyl)-6-Sulfatase/GNS Products
Product Specific Notices
This product is provided under an intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.
For research use only
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