GAA Antikörper (N-Term)

Produktnummer ABIN2797807

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch GAA in WB und IHC. Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für GAA Antikörper (N-Term) (ABIN2797807)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (IHC)

Produktdetails anti-GAA Antikörper

Bindungsspezifität: AA 174-203, N-Term

Verwendungszweck: Rabbit Anti-Human GAA (N-term) Antibody

Immunogen: This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

Isotyp: Ig Fraction

Anwendungsinformationen

Applikationshinweise: Western Blot, Immunohistochemistry
Recommended Dilutions
WB: 1:1000, IHC: 1:10-50GAA Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 0.460 mg/mL

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 2-8°C (short-term), -20°C (long-term)

Details zu GAA

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

Andere Bezeichnung: GAA

Hintergrund:

Target Description: 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.

Gene Symbol: GAA

Molekulargewicht: 105324 Da

Gen-ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process