GBE1 Antikörper (AA 79-515)

Produktnummer ABIN7602464

Dieses Anti-GBE1-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von GBE1 in WB und ELISA. Geeignet für Human, Maus und Ratte.

Kurzübersicht für GBE1 Antikörper (AA 79-515) (ABIN7602464)

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: Anti-GBE1 Antibody Picoband®

Produktmerkmale: Anti-GBE1 Antibody Picoband® (ABIN7602464). Tested in WB, ELISA applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Aufreinigung: Immunogen affinity purified.

Immunogen: E.coli-derived human GBE1 recombinant protein (Position: H79-R515). Human GBE1 shares 92.4% amino acid (aa) sequence identity with mouse GBE1.

Anwendungsinformationen

Applikationshinweise: Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
ELISA, 0.1-0.5 μg/mL, -
1. Akman, H. O., Emmanuele, V., Kurt, Y. G., Kurt, B., Sheiko, T., DiMauro, S., Craigen, W. J. A novel mouse model that recapitulates adult-onset glycogenosis type 4. Hum. Molec. Genet. 24: 6801-6810, 2015. 2. Akman, H. O., Kakhlon, O., Coku, J., Peverelli, L., Rosenmann, H., Rozenstein-Tsalkovich, L., Turnbull, J., Meiner, V., Chama, L., Lerer, I., Shpitzen, S., Leitersdorf, E., Paradas, C., Wallace, M., Schiffmann, R., DiMauro, S., Lossos, A., Minassian, B. A. Deep intronic GBE1 mutation in manifesting heterozygous patients with adult polyglucosan body disease. JAMA Neurol. 72: 441-445, 2015. Note: Erratum: JAMA Neurol. 72: 481 only, 2015. 3. Alegria, A., Martins, E., Dias, M., Cunha, A., Cardoso, M. L., Maire, I. Glycogen storage disease type IV presenting as hydrops fetalis. J. Inherit. Metab. Dis. 22: 330-332, 1999.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Adding 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.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Details zu GBE1

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

Andere Bezeichnung: GBE1

Hintergrund: 1,4-alpha-glucan-branching enzyme, also known as brancher enzyme or glycogen-branching enzyme is an enzyme that in humans is encoded by the GBE1 gene. The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease).

Molekulargewicht: 80 kDa

Gen-ID: 2632

UniProt: Q04446

Pathways: Cellular Glucan Metabolic Process