GNS antibody (AA 1-100) (AbBy Fluor® 647)

Catalog No. ABIN1699066

Product Details anti-GNS Antibody (AbBy Fluor® 647)

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

Binding Specificity: AA 1-100

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GNS antibody is conjugated to AbBy Fluor® 647

Application: Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Predicted Reactivity: Human,Mouse,Rat,Cow,Horse,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for GNS

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

Alternative Name: Glucosamine 6 sulfatase (GNS Products)

Synonyms: G6S antibody, 2610016K11Rik antibody, AU042285 antibody, C87209 antibody, N28088 antibody, NV14559 antibody, N-acetylglucosamine-6-sulfatase antibody, zgc:114066 antibody, gns antibody, wu:fi20h10 antibody, zgc:55370 antibody, glucosamine (N-acetyl)-6-sulfatase antibody, glucosamine (N-acetyl)-6-sulfatase S homeolog antibody, glucosamine (N-acetyl)-6-sulfatase a antibody, N-acetylglucosamine-6-sulfatase antibody, glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), b antibody, GNS antibody, Gns antibody, gns.S antibody, gns antibody, gnsa antibody, CpipJ_CPIJ000745 antibody, Sros_7372 antibody, VDBG_04409 antibody, Halhy_3165 antibody, gnsb antibody

Background:

Synonyms: 2610016K11Rik, AU042285, C87209, G6S, Glucosamine N-acetyl 6 sulfatase, Glucosamine 6 sulfatase, Glucosamine-6-sulfatase, GNS, GNS_HUMAN, MGC21274, N acetylglucosamine 6 sulfatase [Precursor], N-acetylglucosamine-6-sulfatase, N28088.

Background: GNS is a 552 amino acid lysosomal enzyme that hydrolyzes the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate and heparan sulfate. A member of the sulfatase family, GNS assists in the catabolism of heparin, and binds calcium as a cofactor. GNS deficiency results in an autosomal recessive lysosomal storage disorder known as mucopolysaccharidosis type IIID (Sanfilippo D syndrome), which is characterized by mild somatic disease and severe degeneration of the central nervous system. Subject to post-translational internal peptidase cleavage, GNS is encoded by a gene mapping to human chromosome 12q14.2 and mouse chromosome 10 D2.

Gene ID: 2799

Pathways: Glycosaminoglycan Metabolic Process