GBE1 Antibody, Cy7 Conjugated
GBE1 Antibody, Cy7 Conjugated is available 1 time from Bioss primary conjugated antibodies labs
bs-13300R-Cy7 | GBE1 Antibody, Cy7 Conjugatedsize: 0.1ml | 396.24 USD
- Long nameGBE1 Polyclonal Antibody, Cy7 Conjugated
- CategoryConjugated Primary Antibodies
- Host OrganismRabbit (Oryctolagus cuniculus)
- Target AntigenGBE1
- SpecificityThis is a highly specific antibody against GBE1.
- Modification siteNone
- ClonePolyclonal antibody
- Concentration1ug per 1ul
- SourceKLH conjugated synthetic peptide derived from human GBE1
- Gene ID number2632
- Tested applicationsIF(IHC-P)
- Recommended dilutionsIF(IHC-P)(1:50-200)
- CrossreactivityHuman, Mouse, Rat
- Crossreactive species detailsDue to limited amount of testing and knowledge, not every possible cross-reactivity is known.
- Antigen backgroundGBE1 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 methodThis antibody was purified via Protein A.
- Storage conditionsKeep 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 Emission743nm/767nm
- Synonyms1,4 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.
- Gene target
- Short nameGBE1 Antibody, Conjugated
- TechniqueAntibody, antibodies against human proteins, antibodies for, antibody Conjugates
- Alternative nameGBE1 (Antibody to), cyanine 7 coupled
- Alternative techniqueantibodies
Supplier:Bioss Polyclonal Antibodies