| Long name: |
GBE1 Primary Polyclonal Antibody |
| Also known as: |
GBE1 PAb |
| Category: |
Primary Antibodies |
| Conjugation: |
Unconjugated |
| Target Antigen: |
GBE1 |
| Specificity: |
This is a highly specific antibody against GBE1 |
| Modification(s): |
None |
| Modification site(s): |
Unmodified antibody |
| Clonality: |
Polyclonal antibody |
| Clone number: |
Polyclonal antibody |
| Concentration: |
1ug per 1ul |
| Source: |
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human GBE1 |
| Gene ID number: |
2632 |
| Tested Applications: |
IF(IHC-P), IHC-P, WB |
| Recommended dilutions: |
IF(IHC-P)(1:50-200), IHC-P(1:100-500), WB(1:100-1000) |
| Cross reactivity: |
Mouse, Rat, Human |
| Cross reactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Background of the target antigen: |
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 at -20°, 09% sodium azide, C for up to 1 year, Keep the antibody in aqueous buffered solution containing 1% BSA |
| Synonym names: |
4-alpha-glucan-branching enzyme, Andersen disease, Brancher enzyme, GBE, GBE 1, GBE1, GLGB_HUMAN, Glucan 1, Glycogen branching enzyme, Glycogen storage disease type IV, Glycogen-branching enzyme, OTTHUMP00000213788, OTTHUMP00000213833, amylo 1, amylo 1, branching enzyme 1, gGlucan 1, 1, 4 alpha , 4 alpha branching enzyme, 4 alpha glucan branching enzyme, 4 to 1, 4 to 1, 6 transglucosidase, 6 transglycosylase |
| Properties: |
C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24° |
| Gene target: |
GBE1 |
| Short name: |
GBE1 Antibody |
| Technique: |
antibodies against human proteins, antibodies for, Antibody |
| Alternative name: |
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 |