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Long name
GBE1 Polyclonal Antibody, ALEXA FLUOR 647 Conjugated
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Also known as
Anti-GBE1 PAb ALEXA FLUOR 647
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Category
Conjugated Primary Antibodies
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Conjugated with
ALEXA FLUOR® 647
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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
This antibody was obtained by immunization of the host with 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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Cross reactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Background of the antigen
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
Purified by Protein A.
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Storage conditions
Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
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Excitation emission
650nm/665nm
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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
For facs or microscopy Alexa 1 conjugate. Alexa Fluor 633 is a practical alternative to APC as well as Cy5. Bioss Primary Conjugated Antibodies. ALEXA FLUOR made this Alexa Fluor 633 conjugate that can be used in multi-color flow cytometry with instruments equipped with a second red laser or red diode. It is detected in the FL4 detector of the core's upgraded 2-laser FACScans. Like other Alexa Fluor dyes, the Anti-GBE1 exhibits uncommon photo stability, making it an ideal choice for fluorescent microscopy. If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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Conjugation
Alexa Fluor, ALEXA FLUOR 647
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French translation
anticorps
