GBE1 Antibody, FITC Conjugated
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Catalog number
bs-13300R-FITC
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Price
Please ask
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Size
0.1ml
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Long name
GBE1 Polyclonal Antibody, FITC Conjugated
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Category
Conjugated Primary Antibodies
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Conjugation
FITC
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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
494nm/518nm
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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. This Bioss Primary Conjugated Antibodies Fluorescein isothiocyanate (FITC) antibody is currently after some BD antibodies the most commonly used fluorescent dye for FACS. When excited at 488 nanometers, FITC has a green emission that's usually collected at 530 nanometers, the FL1 detector of a FACSCalibur or FACScan. FITC has a high quantum yield (efficiency of energy transfer from absorption to emission fluorescence) and approximately half of the absorbed photons are emitted as fluorescent light. For fluorescent microscopy applications, the 1 FITC is seldom used as it photo bleaches rather quickly though in flow cytometry applications, its photo bleaching effects are not observed due to a very brief interaction at the laser intercept. Bioss Primary Conjugated Antibodies FITC is highly sensitive to pH extremes.
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Additional conjugation
Anti-FITC Antibody
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French translation
anticorps
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Gene target
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Gene symbol
GBE1
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Short name
Anti-GBE1
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Technique
Antibody, FITC, antibodies against human proteins, antibodies for, antibody Conjugates, Fluorescein
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Isotype
Immunoglobulin G (IgG)
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Label
FITC
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Alternative name
Anti-GBE1 PAb FITC
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Alternative technique
antibodies, fluorescine
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Gene info
MeSH Data
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Name
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Concept
Scope note:
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
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Tree numbers
- E01.370.225.500.607.512.240
- E01.370.225.750.551.512.240
- E05.200.500.607.512.240
- E05.200.750.551.512.240
- E05.478.583.375
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Qualifiers
ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data
Product images
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