| Type: |
Conjugated Primary Antibody |
| Conjugated with: |
594, ALEXA FLUOR® |
| Host organism: |
Rabbit (Oryctolagus cuniculus) |
| Target Protein/Peptide: |
GBE1 |
| Specificity: |
This antibody reacts specifically with GBE1 |
| Modification: |
No modification has been applied to this antibody |
| Modification site: |
None |
| Clonality: |
Polyclonal Antibody |
| Clone: |
Polyclonal Antibodies |
| Concentration: |
1ug per 1ul |
| Antigen Source: |
KLH conjugated synthetic peptide derived from human GBE1 |
| Gene ID: |
2632 |
| Applications: |
IF(IHC-P) |
| Applications with corresponding dilutions: |
IF(IHC-P)(1:50-200) |
| Cross reactive species: |
Mouse (Mus musculus), Rat (Rattus norvegicus), Human (Homo sapiens) |
| Cross Reactive Species details: |
However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species, No significant cross reactivity has been observed for this antibody for the tested species |
| Background information: |
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: |
Purified by Protein A |
| Storage: |
50% glycerol and 0, Store at 4°, 09% sodium azide, C for 12 months, Water buffered solution containing 100ug/ml BSA |
| Excitation emission: |
590nm/617nm |
| Also known as: |
GBE1 Polyclonal Antibody |
| Other name: |
Anti-GBE1 Polyclonal |
| Advisory: |
For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube, Prior to use briefly centrifuge the vial to gather all the solution on the bottom, specificity and sensitivity, thus reducing its reactivity, Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody |
| Properties: |
For facs or microscopy Alexa 1 conjugate |
| Conjugation: |
Alexa Fluor |
| Group: |
Polyclonals and antibodies |
| About: |
The advantage is that there are more epitopes available in a polyclonal antiserum to detect the proteins than in monoclonal sera, immunohistochemistry on frozen slices or parrafin fixed tissues, Polyclonals can be used for Western blot |
| Gene target: |
GBE1 |
| Short name: |
Anti-GBE1 (Polyclonal) Fluor 594 |
| Technique: |
Pabs are a collection of immunoglobulin , Polyclonal antibodies , each identifying a different , whereas , (pAbs) are mostly rabbit or goat , B cells, Polyclonal, antibodies , antigen, come from a single N cell lineage, epitope, molecules that react against a specific , monoclonal antibodies , that are secreted by different |
| Label: |
ALEXA |
| Alternative name: |
ALEXA Fluor 594, antibody to-GBE1 (polyclonal) |
| Alternative technique: |
polyclonals |
| 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 |