GBE1 Antikörper (AA 79-515)

Produktnummer ABIN7876802

Dieser Kaninchen Polyklonal Antikörper detektiert spezifisch GBE1 in WB und ELISA. Es zeigt Reaktivität gegenüber Proben von Human, Maus und Ratte.

Kurzübersicht für GBE1 Antikörper (AA 79-515) (ABIN7876802)

Target: GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GBE1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Produktdetails anti-GBE1 Antikörper

Bindungsspezifität: AA 79-515

Verwendungszweck: GBE1 Antibody / 1,4-alpha-glucan branching enzyme 1

Aufreinigung: Immunogen affinity purified

Immunogen: E.coli-derived human GBE1 recombinant protein (Position: H79-R515) was used as the immunogen for the GBE1 antibody.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the GBE1 antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

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

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: After reconstitution, the GBE1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Details zu GBE1

Target: GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

Andere Bezeichnung: GBE1

Hintergrund: GBE1 antibody detects 1,4-alpha-glucan branching enzyme 1, an essential enzyme in glycogen biosynthesis responsible for introducing alpha-1,6 glycosidic branches into linear glucan chains. Encoded by the GBE1 gene on chromosome 3p12.3, this enzyme catalyzes the formation of glycogen's highly branched structure, which enhances solubility and allows rapid synthesis and mobilization of glucose. GBE1 is a crucial metabolic regulator in liver, skeletal muscle, and other tissues involved in energy storage and glucose homeostasis.

Structurally, GBE1 is a 702-amino-acid cytosolic enzyme of approximately 80 kilodaltons that belongs to the glycoside hydrolase family 13. It contains a central catalytic domain with the conserved Asp-Glu-Asp catalytic triad and carbohydrate-binding modules that enable glucan chain recognition and rearrangement. By transferring short alpha-1,4-linked oligosaccharide chains to alpha-1,6 positions, GBE1 generates the branch points that define glycogen's compact and accessible configuration.

The GBE1 antibody is widely used in glycogen metabolism, endocrinology, and neuromuscular research to study carbohydrate storage, enzymatic regulation, and metabolic disease mechanisms. Western blot analysis detects an 80 kilodalton band corresponding to GBE1, while immunofluorescence shows diffuse cytoplasmic staining, particularly in hepatocytes and myocytes. This antibody supports investigations of glycogen biosynthesis and the enzymatic defects underlying glycogen storage disorders.

Mutations in GBE1 cause Glycogen Storage Disease Type IV (Andersen disease) and its non-lethal variant, adult polyglucosan body disease (APBD). These disorders are characterized by abnormal glycogen accumulation and progressive neuromuscular or hepatic dysfunction. Reduced GBE1 activity leads to poorly branched glycogen with decreased solubility and impaired mobilization. The GBE1 antibody provides a reliable tool for studying enzyme expression and activity in models of metabolic disease, helping to elucidate molecular mechanisms that govern glycogen structure and energy regulation. NSJ Bioreagents validates this antibody for its applications, ensuring high specificity and reproducibility in metabolic research.

UniProt: Q04446

Pathways: Cellular Glucan Metabolic Process