Welcome to GenPrice! Check out our latest updates.

Shopping Cart (0)

Your cart is empty

Add some products to get started!

Anti-GBE1 Antibody Picoband® Fluoro488 Conjugated

Product Specifications

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) .

Gene Name

GBE1

Gene ID

2632

UniProt

Q04446

Host

Rabbit

Reactivity

Human, Mouse, Rat

Immunogen

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

Clonality

Polyclonal

Applications

Flow Cytometry

Purification

Immunogen affinity purified.

Form

Liquid

References & Citations

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.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Applications Notes

6

Isotype

IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

Curated Selection

Explore Other Products

Discover premium biology products from our extensive collection of 20M+ items

SLC27A4 Antibody
A76531-100UL 100 µL

SLC27A4 Antibody

Ask
View Details
Siglec 7, CT (H422) (Sialic Acid-binding Ig-like Lectin 7, Siglec 7, QA79 Membrane Protein, Adhesion Inhibitory Receptor Molecule-1, AIRM-1, p75, D-siglec, AIRM1, D-siglec, CDw328, CD328) (MaxLight 650) discontinued
S1013-60D-ML650 100 µL

Siglec 7, CT (H422) (Sialic Acid-binding Ig-like Lectin 7, Siglec 7, QA79 Membrane Protein, Adhesion Inhibitory Receptor Molecule-1, AIRM-1, p75, D-siglec, AIRM1, D-siglec, CDw328, CD328) (MaxLight 650) discontinued

Ask
View Details
Hsc70 (HSPA8) Mouse Monoclonal Antibody [Clone ID: 1F2-H5]
TA326362 200 µg

Hsc70 (HSPA8) Mouse Monoclonal Antibody [Clone ID: 1F2-H5]

Ask
View Details
Human PKA RI alpha Recombinant Protein C-6 His Tag Lyophilized
MBS8433153-01 0.05 mg

Human PKA RI alpha Recombinant Protein C-6 His Tag Lyophilized

Ask
View Details
Human PKA RI alpha Recombinant Protein C-6 His Tag Lyophilized
MBS8433153-02 5x 0.05 mg

Human PKA RI alpha Recombinant Protein C-6 His Tag Lyophilized

Ask
View Details
Culture Medium for Human Gastric Carcinoma Cells+Luc [NCI-N87+Luc]
AFG-ELL-3255-01 125 mL

Culture Medium for Human Gastric Carcinoma Cells+Luc [NCI-N87+Luc]

Ask
View Details