Glucosamine (N-acetyl)-6-Sulfatase/GNS Antibody [Unconjugated] Summary
Immunogen |
Mouse myeloma cell line NS0-derived recombinant human Glucosamine (N-acetyl)‑6-Sulfatase/GNS Val37-Leu552 Accession # P15586 |
Specificity |
Detects human Glucosamine (N-acetyl)-6‑Sulfatase/GNS in direct ELISAs and Western blots. |
Source |
N/A |
Isotype |
IgG |
Clonality |
Polyclonal |
Host |
Goat |
Gene |
GNS |
Purity Statement |
Antigen Affinity-purified |
Innovator's Reward |
Test in a species/application not listed above to receive a full credit towards a future purchase. |
Applications/Dilutions
Dilutions |
- Immunoprecipitation 25 ug/mL
- Western Blot 0.1 ug/mL
|
Packaging, Storage & Formulations
Storage |
Use a manual defrost freezer and avoid repeated freeze-thaw cycles. - 12 months from date of receipt, -20 to -70 °C as supplied.
- 1 month, 2 to 8 °C under sterile conditions after reconstitution.
- 6 months, -20 to -70 °C under sterile conditions after reconstitution.
|
Buffer |
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS. |
Preservative |
No Preservative |
Concentration |
LYOPH |
Reconstitution Instructions |
Reconstitute at 0.2 mg/mL in sterile PBS. |
Notes
This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.
Alternate Names for Glucosamine (N-acetyl)-6-Sulfatase/GNS Antibody [Unconjugated]
Background
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.
- 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 York, McGraw-Hill.
- Robertson, D.A. et al. (1992) Biochem. J. 288:539.
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are
guaranteed for 1 year from date of receipt.
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