Rabbit anti-Human Glucosamine Polyclonal Antibody | anti-G6S antibody

Glucosamine (N-acetyl)-6-Sulfatase (Glucosamine-6-sulfatase, G6S, GNS, N-acetylglucosamine-6-sulfatase, MGC21274) (MaxLight 750)

Cat. Num. AAA6478020

• Gene Names: GNS; G6S
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: Glucosamine; Polyclonal Antibody; Glucosamine (N-acetyl)-6-Sulfatase (Glucosamine-6-sulfatase; G6S; GNS; N-acetylglucosamine-6-sulfatase; MGC21274) (MaxLight 750); EC=3.1.6.14; anti-G6S antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human GNS.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight750.

• Applicable Applications for anti-G6S antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:50-1:100; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the center region of human GNS (KLH).
• Conjugate: MaxLight750
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight750 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of GNS Antibody (Center S298) in NCI-H460, Hela, 293 cell line lysates (35ug/lane). GNS (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(GNS Antibody (Center S298) IHC analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the GNS Antibody (Center S298) for immunohistochemistry. Clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(GNS Antibody (Center S298) flow cytometric analysis of NCI-H460 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-G6S antibody

Antibodies; Enzymes

References

1. Zhang, H., Li, X.J., Nat. Biotechnol. 21(6), 660-666 (2003).

NCBI and Uniprot Product Information

• NCBI GI #: 4504061
• NCBI GeneID: 2799
• NCBI Accession #: NP_002067
• NCBI GenBank Nucleotide #: NM_002076
• UniProt Accession #: P15586
• Molecular Weight: 62kDa
• NCBI Official Full Name: N-acetylglucosamine-6-sulfatase
• NCBI Official Synonym Full Names: glucosamine (N-acetyl)-6-sulfatase
• NCBI Official Symbol: GNS
• NCBI Official Synonym Symbols: G6S
• NCBI Protein Information: N-acetylglucosamine-6-sulfatase
• UniProt Protein Name: N-acetylglucosamine-6-sulfatase
• Protein Family: Glucosamine kinase
• UniProt Gene Name: GNS
• UniProt Synonym Gene Names: G6S
• UniProt Entry Name: GNS_HUMAN

NCBI Description

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. [provided by RefSeq, Jul 2008]

Uniprot Description

GNS: Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D); also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. Belongs to the sulfatase family.

Protein type: EC 3.1.6.14; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation

Chromosomal Location of Human Ortholog: 12q14

Cellular Component: lysosomal lumen

Molecular Function: protein binding; N-acetylglucosamine-6-sulfatase activity; sulfuric ester hydrolase activity; metal ion binding

Biological Process: keratan sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; keratan sulfate catabolic process

Disease: Mucopolysaccharidosis, Type Iiid