GBE1 Antibody, Biotin Conjugated
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Catalog number
bs-13300R-Biotin
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Price
Please ask
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Size
0.1ml
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Long name
GBE1 Polyclonal Antibody, Biotin Conjugated
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Category
Conjugated Primary Antibodies
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Conjugation
Biotin
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Host Organism
Rabbit (Oryctolagus cuniculus)
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Target Antigen
GBE1
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Specificity
This is a highly specific antibody against GBE1.
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Modification
Unmodified
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Modification site
None
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Clonality
Polyclonal
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Clone
Polyclonal antibody
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Concentration
1ug per 1ul
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Source
KLH conjugated synthetic peptide derived from human GBE1
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Gene ID number
2632
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Tested applications
WB, IHC-P
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Recommended dilutions
WB(1:100-1000), IHC-P(1:100-500)
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Crossreactivity
Human, Mouse, Rat
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Crossreactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Antigen 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.
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Purification method
This antibody was purified via Protein A.
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Storage conditions
Keep the antibody in an aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store refrigerated at 2 to 8 degrees Celcius for up to 1 year.
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Excitation Emission
N/A
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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.
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Properties
If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C. Biotin conjugates can be detected by horseradish peroxidase, alkaline phosphatase substrates or anti biotin conjugated antibodies. Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA. To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1.6.
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Additional conjugation
Biotinylated
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French translation
anticorps
Gene info
MeSH Data
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Name
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Concept
Scope note:
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
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Tree numbers
- E05.196.401.143
- E05.301.300.096
- E05.478.566.320.200
- E05.601.262
- E05.601.470.320.200
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Qualifiers
ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data
Product images
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