| Long name: |
Biotin Conjugated, GBE1 Polyclonal Antibody |
| Category: |
Conjugated Primary Antibodies |
| Conjugation: |
Biotinylated |
| Host Organism: |
Rabbit (Oryctolagus cuniculus) |
| Target Antigen: |
GBE1 |
| Specificity: |
This is a highly specific antibody against GBE1 |
| Modification: |
Unmodified |
| Modification site: |
None |
| Clonality: |
Polyclonal |
| Clone: |
Polyclonal antibody |
| Concentration: |
1ug per 1ul |
| Source: |
KLH conjugated synthetic peptide derived from human GBE1 |
| Gene ID number: |
2632 |
| Tested applications: |
IHC-P, WB |
| Recommended dilutions: |
IHC-P(1:100-500), WB(1:100-1000) |
| Crossreactivity: |
Mouse, Rat, Human |
| Crossreactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Antigen background: |
APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), Existing as a monomer, GBE1 catalyzes the transfer of alpha-1, Unlike GSD4, plays an essential role in glycogen accumulation, pyramidal tetraparesis and peripheral neuropathy, the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood, via this catalytic activity, 4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1, 6 position on a neighboring glycogen chain and, GBE1 is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis |
| Purification method: |
This antibody was purified via Protein A |
| Storage conditions: |
50% glycerol and 0, Store refrigerated at 2 to 8 degrees Celcius for up to 1 year, 09% sodium azide, Keep the antibody in an aqueous buffered solution containing 1% BSA |
| Properties: |
Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA, Biotin conjugates can be detected by horseradish peroxidase, To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1, alkaline phosphatase substrates or anti biotin conjugated antibodies, 6, C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24° |
| Gene target: |
GBE1 Conjugated |
| Short name: |
Conjugated, GBE1 Antibody |
| Technique: |
antibodies against human proteins, antibodies for, antibody Conjugates, Antibody |
| Label: |
Biotin |
| Alternative name: |
biotinilated coupled, GBE1 (Antibody to) |
| Alternative technique: |
antibodies |
| Identity: |
4180 |
| Gene: |
GBE1 |
More about : GBE1 |
| Long gene name: |
1, 4-alpha-glucan branching enzyme 1 |
| Synonyms gene name: |
branching enzyme 1 , glucan (1, 4-alpha-) |
| Synonyms name: |
glycogen branching enzyme Andersen disease glycogen storage disease type IV |
| Locus: |
3p12, 2 |
| Discovery year: |
1993-06-21 |
| Entrez gene record: |
2632 |
| Pubmed identfication: |
8463281 |
| Havana BLAST/BLAT: |
OTTHUMG00000158978 |