Specifications
- Antibody type:Primary
- Antigen name:GBE1
- Clonality:Polyclonal
- Conjugation:Alexa Fluor® 680
- Host:Rabbit
- ImmunoChemistry:Yes
- ImmunoFluorescence:Yes
- Isotype:IgG
- Western blot:Yes
- Epitope:101-200/702
- Form:Liquid
- Gene ID:2632
- Antigen synonyms:Glycogen branching enzyme|GLGB_HUMAN|amylo 1,4 to 1,6 transglycosylase|OTTHUMP00000213788|GBE|Glycogen storage disease type IV|amylo 1,4 to 1,6 transglucosidase|Brancher enzyme|Andersen disease|Glucan 1,4 alpha branching enzyme|GBE1|GBE 1|1,4 alpha glucan branching enzyme|Glycogen-branching enzyme|OTTHUMP00000213833|4-alpha-glucan-branching enzyme|gGlucan 1,4 alpha , branching enzyme 1
- Modification:Unmodified
- Storage buffer:Aqueous buffered solution containing 0,01M TBS (pH 7,4) with 1% BSA, 0,03% Proclin300 and 50% Glycerol.
- Molecular weight:80 kDa
- Storage temperature:Store at −20 °C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Concentration:1 µg/µl
- Shipping temperature:4 °C
- Immunogen:KLH conjugated synthetic peptide derived from human GBE1
- Tested applications:ICC
- Purification:Purified by Protein A
- Pack type:Vial
- Pk:100 µl
Specifications
About this item
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.
Type: Primary
Antigen: GBE1
Clonality: Polyclonal
Clone:
Conjugation: ALEXA FLUOR® 680
Public Immunogen Range: 101-200/702
Host: Rabbit
Isotype: IgG
Reactivity: