GAA Antikörper (AA 494-527)

Produktnummer ABIN5647392

Produktdetails anti-GAA Antikörper

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

Bindungsspezifität: AA 494-527

Reaktivität: Human, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

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

Aufreinigung: Antigen affinity purified

Immunogen: Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the GAA antibody should be determined by the researcher.\. Western blot: 0.5-1 μg/mL,Immunohistochemistry (FFPE): 1-2 μg/mL

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Buffer: 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

Lagerung: -20 °C

Informationen zur Lagerung: After reconstitution, the GAA antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Details zu GAA

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

Andere Bezeichnung: GAA / Glucosidase alpha acid (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: Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process