GBE1 polyclonal antibody

CAT: 0384-BS8283-01Size: 50 µLDry Ice: NoHazardous: No
CAT#:0384-BS8283-01Size:50 µL
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Background
GBE1 (glucan (1,4-alpha-), branching enzyme 1) 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.
CAS Number
9007-83-4
Structure Composition
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Product Name Alternative
1,4-alpha-glucan-branching enzyme; Brancher enzyme; Glycogen-branching enzyme; GBE1
Swiss Prot
Q04446
Reactivity
Human, Mouse, Rat
Immunogen
Recombinant full length Human GBE1.
Conjugation
Unconjugated
Applications
WB
Dilution
WB 1:500 - 1:2000
Purity
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Modification
Unmodification
Molecular Weight
~ 80 kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Notes
For research use only, not for use in diagnostic procedure.
Specificity
GBE1 polyclonal antibody detects endogenous levels of GBE1 protein.
Applications Notes
WesternBlot (WB) analysis of GBE1 polyclonal antibody
Host or Source
Rabbit

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