GNS antibody (AA 283-313)

Catalog No. ABIN390493

Product Details anti-GNS Antibody

Target: GNS (Glucosamine (N-Acetyl)-6-Sulfatase (GNS))

Binding Specificity: AA 283-313

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GNS antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Flow Cytometry (FACS)

Purification: This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Immunogen: This GNS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 283-313 amino acids from the Central region of human GNS.

Clone: RB19341

Isotype: Ig Fraction

Application Details

Application Notes: WB: 1:1000. IHC-P: 1:50~100. FC: 1:10~50

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Expiry Date: 6 months

Target Details for GNS

Target: GNS (Glucosamine (N-Acetyl)-6-Sulfatase (GNS))

Alternative Name: GNS (GNS Products)

Background: GNS is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.

Molecular Weight: 62082

Gene ID: 2799

NCBI Accession: NP_002067

UniProt: P15586

Pathways: Glycosaminoglycan Metabolic Process