GAA Antikörper

Produktnummer ABIN7263675

Produktdetails anti-GAA Antikörper

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

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Produktmerkmale: Polyclonal Antibody

Aufreinigung: Affinity purification

Immunogen: A synthetic peptide of human GAA (NP_000143.2).

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:500-1:2000

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 mg/mL

Buffer: PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3

Konservierungsmittel: Sodium azide

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Avoid freeze / thaw cycles.

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: This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

Molekulargewicht:

Observed_MW: 105 kDa

Calculated_MW: 105 kDa

Gen-ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process