Long name GBE1 Polyclonal Antibody, Cy7 Conjugated
Category Conjugated Primary Antibodies
Host Organism Rabbit (Oryctolagus cuniculus)
Target Antigen GBE1
Specificity This is a highly specific antibody against GBE1.
Modification site None
Clone Polyclonal antibody
Concentration 1ug per 1ul
Source KLH conjugated synthetic peptide derived from human GBE1
Gene ID number 2632
Tested applications IF(IHC-P)
Recommended dilutions IF(IHC-P)(1:50-200)
Crossreactivity Human, Mouse, Rat
Crossreactive species details Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
Antigen 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.
Purification method This antibody was purified via Protein A.
Storage conditions Keep the antibody in an aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store refrigerated at 2 to 8 degrees Celcius for up to 1 year.
Excitation Emission 743nm/767nm
Synonyms 1,4 alpha glucan branching enzyme; 4-alpha-glucan-branching enzyme; amylo 1,4 to 1,6 transglucosidase; amylo 1,4 to 1,6 transglycosylase; Andersen disease; Brancher enzyme; GBE 1; GBE; GBE1; gGlucan 1,4 alpha , branching enzyme 1; GLGB_HUMAN; Glucan 1,4 alpha branching enzyme; Glycogen branching enzyme; Glycogen storage disease type IV; Glycogen-branching enzyme; OTTHUMP00000213788; OTTHUMP00000213833.
Short name GBE1 Antibody, Conjugated
Technique Antibody, antibodies against human proteins, antibodies for, antibody Conjugates
Alternative name GBE1 (Antibody to), cyanine 7 coupled
Alternative technique antibodies