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The antibody against GBE1 was raised in rabbit using the Recombinant Human 1,4-alpha-glucan-branching enzyme protein (1-300aa) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, IHC.
The antibody against GBE1 was raised in rabbit using the Recombinant Human 1,4-alpha-glucan-branching enzyme protein (1-300aa) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, IHC.
$299.00
| Cat.No | ADC-55110A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | GBE1 |
| Target Synonyms | 1, 4-alpha-glucan-branching enzyme (EC 2.4.1.18) (Brancher enzyme) (Glycogen-branching enzyme), GBE1 | Form | Liquid |
| Species Reactivity | Human | Isotype | IgG |
| Storage Buffer | 0.01M PBS, 0.03% Proclin 300; Constituents: 50% Glycerol, PH 7.4 | Purification Method | >95%, Protein G purified |
| Conjugate | Non-conjugated | Application | ELISA, IHC |
| Storage | Upon receipt |
| Immunogen Description | Recombinant Human 1,4-alpha-glucan-branching enzyme protein (1-300AA) | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | Q04446 |
Uniprot Id
Q04446
Target Species
Human
Target Name
GBE1
Target Full Name
1,4-alpha-glucan-branching enzyme
Target Function
Required for normal glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule.
Target Involvement
Glycogen storage disease 4 (GSD4); Polyglucosan body neuropathy, adult form (APBN)
Target Protein Families
Glycosyl hydrolase 13 family, GlgB subfamily
Target Synonyms
GBE; APBD; GSD4; GBE1
Target Background
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).
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