1-832-868-1888
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Catalog Number: |
39035 |
| other_names: | GBE; APBD; GSD4; |
Amount: |
100μg |
| calculated_mw: | |
| host_species: | Rabbit |
Price: |
$319 |
Swiss-Prot No: |
Swiss-Prot#: Q04446 |
Form of Antibody: |
Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
Storage/Stability: |
|
Immunogen: |
Recombinant protein of human GBE1. |
Purification: |
Antibodies were purified by affinity purification using immunogen. |
Specificity/Sensitivity: |
The antibody detects endogenous level of total GBE1 antibody. |
Applications: |
WB |
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). |
References: |
|
appl_detail: |
WB 1:500 - 1:2000 |
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