GAA Antikörper (AA 541-640)

Produktnummer ABIN1714619

Produktdetails anti-GAA Antikörper

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Bindungsspezifität: AA 541-640

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA, Immunocytochemistry (ICC), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Kreuzreaktivität: Human, Maus, Ratte

Homologie: Dog,Sheep,Pig,Horse

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu GAA

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Andere Bezeichnung: GAA (GAA Produkte)

Synonyme: LYAG antikoerper, E430018M07Rik antikoerper, glucosidase alpha, acid antikoerper, glucosidase, alpha, acid antikoerper, transmembrane and coiled-coil domain family 1 antikoerper, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antikoerper, GAA antikoerper, Gaa antikoerper, TMCC1 antikoerper, gaa antikoerper

Hintergrund:

Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid Pompe disease glycogen storage disease type II, Glucosidase alpha acid, Glucosidase alpha, LYAG, LYAG_HUMAN, Lysosomal alpha glucosidase.

Background: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008].

Gen-ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process