Filters

GBE1 Antibody

GBE1 Antibody size: 0.1ml 263

Supplier BIOSS PRIMARY UNCONJUGATED ANTIBODIES
Price 263
Size 0.1ml
Contact us Ask Component cell markers and DNA bp. markers are genetic markerser genes or DNA sequenceswith selection audit
Long nameGBE1 Primary Polyclonal Antibody
Also known asGBE1 PAb
CategoryPrimary Antibodies
ConjugationUnconjugated
Target AntigenGBE1
SpecificityThis is a highly specific antibody against GBE1
Modification(s)None
Modification site(s)Unmodified antibody
ClonalityPolyclonal antibody
Clone numberPolyclonal 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 Applications IF(IHC-P), IHC-P, WB
Recommended dilutions IF(IHC-P)(1:50-200), IHC-P(1:100-500), WB(1:100-1000)
Cross reactivity 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 target 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
Purification methodThis antibody was purified via Protein A
Storage conditions 50% glycerol and 0, Store at -20°, 09% sodium azide, C for up to 1 year, Keep the antibody in aqueous buffered solution containing 1% BSA
Synonym names 4-alpha-glucan-branching enzyme, Andersen disease, Brancher enzyme, GBE, GBE 1, GBE1, GLGB_HUMAN, Glucan 1, Glycogen branching enzyme, Glycogen storage disease type IV, Glycogen-branching enzyme, OTTHUMP00000213788, OTTHUMP00000213833, amylo 1, amylo 1, branching enzyme 1, gGlucan 1, 1, 4 alpha , 4 alpha branching enzyme, 4 alpha glucan branching enzyme, 4 to 1, 4 to 1, 6 transglucosidase, 6 transglycosylase
PropertiesC, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24°
Gene targetGBE1
Short nameGBE1 Antibody
Technique antibodies against human proteins, antibodies for, Antibody
Alternative nameGBE1 (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

Similar products

Subscribe to our Newsletter