GNS Antibody - C-terminal region

Product Overview

• Article Name: GNS Antibody - C-terminal region
• Biozol Catalog Number: ASB-ARP45217_P050
• Supplier Catalog Number: ARP45217_P050
• Alternative Catalog Number: ASB-ARP45217_P050-25UL,ASB-ARP45217_P050-100UL
• Manufacturer: Aviva
• Host: Rabbit
• Category: Proteine/Peptide
• Application: IHC, WB
• Species Reactivity: Bovine, Canine, Equine, Goat, Guinea pig, Human, Mouse, Rabbit, Rat, Zebrafish
• Immunogen: The immunogen is a synthetic peptide directed towards the C terminal region of human GNS
• Alternative Names: G6S

Product Description

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.

Product Properties

• Clonality: Polyclonal
• Concentration: 0.5 mg/ml
• Molecular Weight: 62kDa
• NCBI: 2799
• UniProt: P15586
• Form: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Images

Rabbit Anti-GNS Antibody
Catalog Number: ARP45217_P050
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

Rabbit Anti-GNS Antibody
Catalog Number: ARP45217_P050
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

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

GNS antibody - C-terminal region (ARP45217_P050) in Human Liver using Western Blot