GNS Rabbit Polyclonal Antibody

CAT#: TA356470

GNS Antibody - C-terminal region

Product Images

WB Suggested Anti-GNS Antibody Titration: 0.2-1 ug/ml ELISA Titer: 1:312500 Positive Control: Human Liver

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  Rabbit Anti-GNS Antibody Catalog Number: TA356470 Formalin Fixed Paraffin Embedded Tissue: Human Liver Tissue Observed Staining: Cytoplasm in Kupffer cells in sinusoids Primary Antibody Concentration: 1:100 Other Working Concentrations: N/A Secondary Antibody: Donkey anti-Rabbit-Cy3 Secondary Antibody Concentration: 1:200 Magnification: 20X Exposure Time: 0.5 - 2.0 sec

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  Rabbit Anti-GNS Antibody Catalog Number: TA356470 Formalin Fixed Paraffin Embedded Tissue: Human heart Tissue Observed Staining: Cytoplasmic Primary Antibody Concentration: 1:100 Other Working Concentrations: N/A Secondary Antibody: Donkey anti-Rabbit-Cy3 Secondary Antibody Concentration: 1:200 Magnification: 20X Exposure Time: 0.5 - 2.0 sec

Specifications

Product Data

• Applications: IHC, WB
• Reactivities: Human
• Host: Rabbit
• Clonality: Polyclonal
• Immunogen: The immunogen is a synthetic peptide directed towards the C terminal region of human GNS
• Specificity: Expected reactivity: Cow, Dog, Goat, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish
  Homology: Cow: 100%; Dog: 93%; Goat: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Zebrafish: 85%
• Formulation: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Concentration: lot specific
• Purification: Affinity Purified
• Conjugation: Unconjugated
• Storage: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
• Predicted Protein Size: 62kDa
• Gene Name: glucosamine (N-acetyl)-6-sulfatase
• Database Link:
  NP_002067
  Entrez Gene 2799 Human
  UniProt ID P15586
• Background: GNS is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparin sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder ucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.The product of this gene 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.
• Synonyms: G6S; Glucosamine-6-sulfatase; MGC21274; N-acetylglucosamine-6-sulfatase

Reference Data

• Protein Families: Druggable Genome, Transmembrane
• Protein Pathways: Glycosaminoglycan degradation, Lysosome, Metabolic pathways