GBE1 anticorps (AA 79-515)

N° du produit ABIN7602464

Cet anticorps anti-GBE1 est un anticorps Lapin Polyclonal détectant GBE1 dans WB et ELISA. Adapté pour Humain, Souris et Rat.

Aperçu rapide pour GBE1 anticorps (AA 79-515) (ABIN7602464)

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

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GBE1 est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-GBE1 anticorps

Épitope: AA 79-515

Fonction: Anti-GBE1 Antibody Picoband®

Attributs du produit: 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.

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

Information d'application

Indications d'application: 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

Stockage

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.

Stock: 4 °C,-20 °C

Stockage commentaire: 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.

Détails sur GBE1

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

Autre désignation: GBE1

Sujet: 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).

Poids moléculaire: 80 kDa

ID gène: 2632

UniProt: Q04446

Pathways: Cellular Glucan Metabolic Process