| Product Name | GBE1 Antibody (F-20) |
|---|---|
| Antibody Type | Primary Antibodies |
| Modification Notes | GBE1 (glucan (1,4-alpha-), branching enzyme 1) is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 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 a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIFELISA |
| recommended for detection of GBE1 of mouse, rat and human origin by WB, IF and ELISA also reactive with additional species, including equine and canine | |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope mapping within an internal region of GBE1 of human origin |
|---|---|
| Tissue Specificity | epitope mapping within an internal region of GBE1 of human origin |
| Entrez Gene | 2632 |
|---|
| Application Notes | recommended for detection of GBE1 of mouse, rat and human origin by WB, IF and ELISA also reactive with additional species, including equine and canine |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
