DATASHEET
Host:
Rabbit
Target Protein:
GBE1
Immunogen Range:
101-200/702
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
2632
Source:
KLH conjugated synthetic peptide derived from human GBE1
Purification:
Purified by Protein A.
Storage Buffer:
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Background:
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.
Conjugation:
PE
Excitation/ Emission:
496,564nm/578nm
Size:
100ul
Concentration:
1ug/ul
Applications:
WB(1:300-5000)
80
Predicted Cross Reactive Species:
Human
Mouse
Rat
Rabbit
For research use only. Not intended for diagnostic or therapeutic use.