SGSH Antikörper (AA 301-388) (AbBy Fluor® 594)

Produktnummer ABIN2806331

Produktdetails anti-SGSH Antikörper (AbBy Fluor® 594)

Target: SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

Bindungsspezifität: AA 301-388

Reaktivität: Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SGSH Antikörper ist konjugiert mit AbBy Fluor® 594

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

Kreuzreaktivität: Ratte

Homologie: Human,Mouse,Dog

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Sulphamidase

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 SGSH

Target: SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

Andere Bezeichnung: SGSH/Sulphamidase (SGSH Produkte)

Synonyme: HSS antikoerper, MPS3A antikoerper, SFMD antikoerper, 4632406A19Rik antikoerper, N-sulfoglucosamine sulfohydrolase antikoerper, N-sulfoglucosamine sulfohydrolase (sulfamidase) antikoerper, SGSH antikoerper, Sgsh antikoerper

Hintergrund:

Synonyms: HSS, SFMD, MPS3A, N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH

Background: Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.

Gen-ID: 6448

UniProt: P51688

Pathways: Glycosaminoglycan Metabolic Process