Rabbit anti-Human GNS Polyclonal Antibody | anti-GNS antibody

GNS (Glucosamine (N-acetyl)-6-sulfatase, G6S, MGC21274) (Biotin)

Cat. Num. AAA6451027

• Gene Names: GNS; G6S
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified
• Synonyms: GNS; Polyclonal Antibody; GNS (Glucosamine (N-acetyl)-6-sulfatase; G6S; MGC21274) (Biotin); Glucosamine (N-acetyl)-6-sulfatase; MGC21274; anti-GNS antibody

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

• Applicable Applications for anti-GNS antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: GNS (NP_002067.1, 1aa-552aa) full-length human protein.
• Immunogen Sequence: MRLLPLAPGRLRRGSPRHLPSCSPALLLLVLGGCLGVFGVAAGTRRPNVVLLLTDDQDEVLGGMTPLKKTKALIGEMGMTFSSAYVPSALCCPSRASILTGKYPHNHHVVNNTLEGNCSSKSWQKIQEPNTFPAILRSMCGYQTFFAGKYLNEYGAPDAGGLEHVPLGWSYWYALEKNSKYYNYTLSINGKARKHGENYSVDYLTDVLANVSLDFLDYKSNFEPFFMMIATPAPHSPWTAAPQYQKAFQNVFAPRNKNFNIHGTNKHWLIRQAKTPMTNSSIQFLDNAFRKRWQTLLSVDDLVEKLVKRLEFTGELNNTYIFYTSDNGYHTGQFSLPIDKRQLYEFDIKVPLLVRGPGIKPNQTSKMLVANIDLGPTILDIAGYDLNKTQMDGMSLLPILRGASNLTWRSDVLVEYQGEGRNVTDPTCPSLSPGVSQCFPDCVCEDAYNNTYACVRTMSALWNLQYCEFDDQEVFVEVYNLTADPDQITNIAKTIDPELLGKMNYRLMMLQSCSGPTCRTPGVFDPGYRFDPRLMFSNRGSVRTRRFSKHLL
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(GNS MaxPab rabbit polyclonal antibody. Western Blot analysis of GNS expression in human liver.)

Western Blot (WB)

(GNS MaxPab rabbit polyclonal antibody. Western Blot analysis of GNS expression in mouse kidney.)

Western Blot (WB)

(Western Blot analysis of GNS expression in transfected 293T cell line by GNS MaxPab polyclonal antibody.Lane 1: GNS transfected lysate(62.1 KDa).Lane 2: Non-transfected lysate.)

Related Product Information for anti-GNS antibody

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]

Product Categories/Family for anti-GNS antibody

Antibodies; Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 4504061
• NCBI GeneID: 2799
• NCBI Accession #: NP_002067.1
• NCBI GenBank Nucleotide #: NM_002076.3
• UniProt Accession #: P15586; Q53F05
• Molecular Weight: 62,082 Da
• 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: Protein
• UniProt Gene Name: GNS
• UniProt Synonym Gene Names: G6S
• UniProt Entry Name: GNS_HUMAN

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