Human Glucosamine (N-acetyl)-6‑Sulfatase/GNS Antibody

Catalog # Availability Size / Price Qty
AF2484
AF2484-SP
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Human Glucosamine (N-acetyl)-6‑Sulfatase/GNS Antibody Summary

Species Reactivity
Human
Specificity
Detects human Glucosamine (N-acetyl)-6‑Sulfatase/GNS in direct ELISAs and Western blots.
Source
Polyclonal Goat IgG
Purification
Antigen Affinity-purified
Immunogen
Mouse myeloma cell line NS0-derived recombinant human Glucosamine (N-acetyl)‑6-Sulfatase/GNS
Val37-Leu552
Accession # P15586
Formulation
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.
Label
Unconjugated

Applications

Recommended Concentration
Sample
Western Blot
0.1 µg/mL
Recombinant Human Glucosamine (N-acetyl)‑6-Sulfatase/GNS (Catalog # 2484-SUC)
Immunoprecipitation
25 µg/mL
Conditioned cell culture medium spiked with Recombinant Human Glucosamine (N-acetyl)‑
6-Sulfatase/GNS (Catalog # 2484-SUC), see our available Western blot detection antibodies

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Reconstitution Calculator

Reconstitution Calculator

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Preparation and Storage

Reconstitution
Reconstitute at 0.2 mg/mL in sterile PBS.
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Shipping
Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.
Stability & 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.

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
  1. Parenti, G. et al. (1997) Curr. Opin. Genet. & Dev. 7:386.
  2. 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.
  3. Robertson, D.A. et al. (1992) Biochem. J. 288:539.
Entrez Gene IDs
2799 (Human)
Alternate Names
EC 3.1.6; EC 3.1.6.14; G6Sglucosamine-6-sulfatase; glucosamine (N-acetyl)-6-sulfatase; Glucosamine6Sulfatase; Glucosamine-6-Sulfatase; GNS; MGC21274; N-acetylglucosamine-6-sulfatase

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