GNS anticorps (AA 1-100) (AbBy Fluor® 350)

N° du produit ABIN1693612

Cet anticorps Lapin Polyclonal détecte spécifiquement GNS dans IF (cc) et IF (p). Il présente une réactivité envers Humain.

Aperçu rapide pour GNS anticorps (AA 1-100) (AbBy Fluor® 350) (ABIN1693612)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GNS est conjugé à/à la AbBy Fluor® 350

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

Détail du produit anti-GNS anticorps (AbBy Fluor® 350)

Épitope: AA 1-100

Homologie: Human,Mouse,Rat,Cow,Horse,Rabbit

Purification: Purified by Protein A.

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

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur GNS

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

Autre désignation: Glucosamine 6 sulfatase

Sujet:

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.

ID gène: 2799

Pathways: Glycosaminoglycan Metabolic Process