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

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  • Long name
    GBE1 Polyclonal Antibody, Cy7 Conjugated
  • Category
    Conjugated Primary Antibodies
  • Conjugation
    Cy7
  • Host Organism
    Rabbit (Oryctolagus cuniculus)
  • Target Antigen
    GBE1
  • Specificity
    This is a highly specific antibody against GBE1.
  • Modification
    Unmodified
  • Modification site
    None
  • Clonality
    Polyclonal
  • 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.
  • Gene target
  • Short name
    GBE1 Antibody, Conjugated
  • Technique
    Antibody, antibodies against human proteins, antibodies for, antibody Conjugates
  • Label
    Cy7
  • Alternative name
    GBE1 (Antibody to), cyanine 7 coupled
  • Alternative technique
    antibodies
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