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HEXB (E9X5S) Mouse Chimeric mAb #87218

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  • IF

    Supporting Data

    REACTIVITY M R
    SENSITIVITY Endogenous
    MW (kDa)
    Source/Isotype Mouse Chimera IgG2a
    Application Key:
    • IF-Immunofluorescence 
    Species Cross-Reactivity Key:
    • M-Mouse 
    • R-Rat 

    Product Information

    Product Description

    This Cell Signaling Technology® antibody retains the antigen-binding Fab regions of the original parent host sequence from which it is engineered. This antibody is expected to exhibit the same species cross-reactivity as HEXB (E9X5S) Rabbit mAb #33663.

    Product Usage Information

    Application Dilution
    Immunofluorescence (Frozen) 1:50 - 1:200

    Storage

    Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

    Protocol

    Specificity / Sensitivity

    HEXB (E9X5S) Mouse Chimeric mAb recognizes endogenous levels of total HEXB protein.

    Species Reactivity:

    Mouse, Rat

    Source / Purification

    This recombinant chimeric antibody is engineered from HEXB (E9X5S) Rabbit mAb #33663 according to animal-free protocols. The chimeric antibody retains its antigen-binding Fab regions from the original rabbit monoclonal antibody, but contains a mouse-derived Fc domain. When multiplexing, Fc-directed rabbit secondaries are required to detect rabbit-host primary antibodies.

    The parent antibody, HEXB (E9X5S) Rabbit mAb #33663, is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Pro300 of mouse HEXB protein.

    Background

    β-hexosaminidase (Hex) is an important lysosomal enzyme system that degrades various cellular substrates, such as oligosaccharides, glycosaminoglycans, and glycolipids. It is encoded by two genes, HEXA and HEXB, respectively, and these subunits dimerize to form β-hexosaminidase A (HexA; αβ) and β-hexosaminidase B (HexB; ββ) (1). Loss-of-function mutations result in ganglioside (GM2) accumulation and progressive neurodegenerative diseases, such as Sandhoff disease (SD) and Tay-Sachs disease (TSD) (1). HexB knockout mice display, similarly to human patients, a near-normal phenotype at birth but quickly develop muscle weakness, rigidity, and motor deterioration, typically leading to death at approximately four months of age (2). It has been shown that loss of HEXB leads to reduction of early neuronal migration and differentiation in the embryonic cerebral cortices of these mice (3). Hex also plays an important role in cancer, being a new potential biomarker in laryngeal cancer. Furthermore, higher expression of HEXA and HEXB is associated with poor prognosis in glioblastoma multiforme (GBM) patients (1).
    For Research Use Only. Not for Use in Diagnostic Procedures.
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