GBE1 antibody (AA 79-515)

Catalog No. ABIN7602464

This anti-GBE1 antibody is a Rabbit Polyclonal antibody detecting GBE1 in WB and ELISA. Suitable for Human, Mouse and Rat.

Quick Overview for GBE1 antibody (AA 79-515) (ABIN7602464)

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

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GBE1 antibody is un-conjugated

Application: Western Blotting (WB), ELISA

Product Details anti-GBE1 Antibody

Binding Specificity: AA 79-515

Purpose: Anti-GBE1 Antibody Picoband®

Characteristics: 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.

Purification: 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.

Application Details

Application Notes: 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.

Restrictions: For Research Use only

Handling

Format: Lyophilized

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

Concentration: 500 μg/mL

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

Storage: 4 °C,-20 °C

Storage Comment: 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.

Target Details for GBE1

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

Alternative Name: GBE1

Background: 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).

Molecular Weight: 80 kDa

Gene ID: 2632

UniProt: Q04446

Pathways: Cellular Glucan Metabolic Process