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The antibody against GBE1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human GBE1 (NP_000149.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
The antibody against GBE1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human GBE1 (NP_000149.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
| Cat.No | ADA-02935A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | GBE1 |
| Target Synonyms | GBE; APBD; GSD4; GBE1 | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.02% sodium azide, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse heart, Mouse kidney, Rat heart, HepG2, Mouse liver, NCI-H460, Rat liver | Application | ELISA, WB |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human GBE1 (NP_000149.3). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q04446 | Immunogen Sequence |
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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