GAA Antikörper

Produktnummer ABIN6989688

Dieses Anti-GAA-Antikörper ist ein Kaninchen Monoklonal-Antikörper zur Detektion von GAA in WB, IHC (p) und FACS. Geeignet für Human.

Kurzübersicht für GAA Antikörper (ABIN6989688)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Monoklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Flow Cytometry (FACS)

Klon: 5H49

Produktdetails anti-GAA Antikörper

Kreuzreaktivität: Human

Aufreinigung: Purified by Protein A.

Immunogen: Synthetic peptide within Human GAA.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
FCM 1:20-100
IHC-P 1:200-400

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 1xTBS ( pH 7.4), 1 % BSA, 40 %Glycerol and 0.05 % Sodium Azide.

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 for 12 months.

Haltbarkeit: 12 months

Details zu GAA

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

Andere Bezeichnung: GAA

Hintergrund:

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

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