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GBE1 Antibody, ALEXA FLUOR 594

GBE1 Antibody, ALEXA FLUOR 594 size: 100ul 332

Supplier BIOSS PRIMARY CONJUGATED ANTIBODIES
Price 332
Size 100ul
Contact us Ask Component cell markers and DNA bp. markers are genetic markerser genes or DNA sequenceswith selection audit
Long name ALEXA FLUOR 594 Conjugated, GBE1 Polyclonal Antibody
Also known asAnti-GBE1 PAb ALEXA FLUOR 594
CategoryConjugated Primary Antibodies
Conjugated with 594, ALEXA FLUOR®
Host OrganismRabbit (Oryctolagus cuniculus)
Target AntigenGBE1
SpecificityThis is a highly specific antibody against GBE1
ModificationUnmodified
Modification SiteNone
ClonalityPolyclonal
ClonePolyclonal antibody
Concentration1ug per 1ul
SourceThis antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human GBE1
Gene ID Number2632
Tested applicationsIF(IHC-P)
Recommended dilutionsIF(IHC-P)(1:50-200)
Crossreactivity 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 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
PurificationPurified by Protein A
Storage conditions 50% glycerol and 0, Keep refrigerated at 2 to 8 degrees Celcius for up to one year, 09% sodium azide, Store this antibody in aqueous buffered solution containing 1% BSA
Excitation emission590nm/617nm
Properties ALEXA FLUOR they should be stored frozen at - 24°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies, C, C for long term storage and for short term at + 5°, For facs or microscopy Alexa 1 conjugate
Conjugation 594, ALEXA FLUOR®, Alexa Fluor
ConjugatedAlexa conjugate 1
Gene targetGBE1
Short nameGBE1 Antibody
Technique antibodies against human proteins, antibodies for, Antibody
LabelALEXA FLUOR 594
Alternative name ALEXA FLUOR 594, GBE1 (Antibody to)
Alternative techniqueantibodies
Identity 4180
Gene 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

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