GAA Antikörper (Middle Region)

Produktnummer ABIN4886596

Produktdetails anti-GAA Antikörper

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

Bindungsspezifität: AA 494-527, Middle Region

Reaktivität: Human, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

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

Verwendungszweck: Rabbit IgG polyclonal antibody for Lysosomal alpha-glucosidase(GAA) detection. Tested with WB, IHC-P in Human,Rat.

Sequenz: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

Kreuzreaktivität (Details): No cross reactivity with other proteins.

Produktmerkmale: Rabbit IgG polyclonal antibody for Lysosomal alpha-glucosidase(GAA) detection. Tested with WB, IHC-P in Human,Rat.
Gene Name: glucosidase alpha, acid
Protein Name: Lysosomal alpha-glucosidase

Aufreinigung: Immunogen affinity purified.

Immunogen: A synthetic peptide corresponding to a sequence in the middle region of human GAA (494-527aa TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR), different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB: Concentration: 0.1-0.5 μg/mL, Tested Species: Human, Rat
IHC-P: Concentration: 0.5-1 μg/mL, Tested Species: Human, Epitope Retrieval by Heat: Boiling the paraffin sections in 10 mM citrate buffer, pH 6.0, for 20 mins is required for the staining of formalin/paraffin sections.
Notes: Tested Species: Species with positive results. Other applications have not been tested. Optimal dilutions should be determined by end users.

Kommentare:

Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Konzentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Sodium azide.

Konservierungsmittel: Sodium azide

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

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: At -20°C for one year. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20 °C for a longer time. Avoid repeated freezing and thawing.

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: 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.

Synonyms: Acid alpha glucosidase | Acid maltase | Aglucosidase alfa | Alpha glucosidase | GAA | Glucosidase alpha acid | Glucosidase alpha | LYAG | P10253

Gen-ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process