GBE1 Polyclonal Antibody, Biotin Conjugated
Applications
Predicted Reactivity
| Overview | |
| Catalog # | bs-13300R-Biotin-100uL |
| Product Name | GBE1 Polyclonal Antibody, Biotin Conjugated |
| Applications |
WB
ELISA
IHC-P
IHC-F
|
| Predicted Reactivity | Human, Mouse, Rat, Rabbit |
| Specifications | |
| Conjugation | Biotin |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human GBE1 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C for 12 months. |
| Target | |
| Gene ID | 2632 |
| Subcellular location | Cytoplasm |
| Synonyms | 1,4 alpha glucan branching enzyme; 4-alpha-glucan-branching enzyme; amylo 1,4 to 1,6 transglucosidase; amylo 1,4 to 1,6 transglycosylase; Andersen disease; Brancher enzyme; GBE 1; GBE; GBE1; gGlucan 1,4 alpha , branching enzyme 1; GLGB_HUMAN; Glucan 1,4 alpha branching enzyme; Glycogen branching enzyme; Glycogen storage disease type IV; Glycogen-branching enzyme; OTTHUMP00000213788; OTTHUMP00000213833. |
| Background | GBE1 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. |
| Application Dilution | |
| WB | 1:300-5000 |
| ELISA | 1:500-1000 |
| IHC-P | 1:200-400 |
| IHC-F | 1:100-500 |
Frequently asked questions
Which applications is GBE1 Polyclonal Antibody, Biotin Conjugated (bs-13300R-Biotin) validated for, and at what dilution?
bs-13300R-Biotin is validated for WB (1:300-5000); ELISA (1:500-1000); IHC-P (1:200-400); IHC-F (1:100-500). These are recommended starting dilutions; optimal dilutions should be determined by the end user.
How should bs-13300R-Biotin be stored?
Store at -20C for 12 months. Storage buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise GBE1 Polyclonal Antibody, Biotin Conjugated (bs-13300R-Biotin)?
KLH conjugated synthetic peptide derived from human GBE1