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

Produktnummer ABIN1699066

Produktdetails anti-GNS Antikörper (AbBy Fluor® 647)

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

Bindungsspezifität: AA 1-100

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GNS Antikörper ist konjugiert mit AbBy Fluor® 647

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

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

Aufreinigung: Purified by Protein A.

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

Isotyp: IgG

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu GNS

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

Andere Bezeichnung: Glucosamine 6 sulfatase (GNS Produkte)

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

Hintergrund:

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.

Gen-ID: 2799

Pathways: Glycosaminoglycan Metabolic Process