GBE1 Antibody, Cy7 Conjugated
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Catalog number
bs-13300R-Cy7
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Price
Please ask
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Size
0.1ml
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Long name
GBE1 Polyclonal Antibody, Cy7 Conjugated
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Category
Conjugated Primary Antibodies
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Conjugation
Cy7
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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
IF(IHC-P)
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Recommended dilutions
IF(IHC-P)(1:50-200)
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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
743nm/767nm
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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.
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Conjugated
These antibodies are excite for emission at 650 nm and detected at a 676 nm wavelengths.
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Additional conjugation
Cy7
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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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