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Render Timestamp: 2024-12-27T12:14:08.981Z
Commit: f2d32940205a64f990b886d724ccee2c9935daff
XML generation date: 2024-09-30 01:59:31.133
Product last modified at: 2024-11-08T16:00:10.713Z
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PDP - Template Name: Monoclonal Antibody
PDP - Template ID: *******c5e4b77
  1. Products /
  2. Primary Antibodies /
  3. HEXB (E9X5S) Rabbit mAb
R Recombinant
Recombinant: Superior lot-to-lot consistency, continuous supply, and animal-free manufacturing.

HEXB (E9X5S) Rabbit mAb #33663

Filter:
  • WB
  • IHC
  • IF

    Supporting Data

    REACTIVITY M R
    SENSITIVITY Endogenous
    MW (kDa) 52, 70
    Source/Isotype Rabbit IgG
    Application Key:
    • WB-Western Blotting 
    • IHC-Immunohistochemistry 
    • IF-Immunofluorescence 
    Species Cross-Reactivity Key:
    • M-Mouse 
    • R-Rat 

    Product Information

    Product Usage Information

    Application Dilution
    Western Blotting 1:1000
    Immunohistochemistry (Paraffin) 1:100 - 1:400
    Immunofluorescence (Frozen) 1:800 - 1:3200
    Immunofluorescence (Immunocytochemistry) 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.

    For a carrier free (BSA and azide free) version of this product see product #28112.

    Protocol

    Specificity / Sensitivity

    HEXB (E9X5S) Rabbit mAb recognizes endogenous levels of total HEXB protein. HEXB (E9X5S) Rabbit mAb #33663 is unable to detect HEXB in some low-expressing cell lines by immunofluorescence.

    Species Reactivity:

    Mouse, Rat

    Source / Purification

    Monoclonal antibody 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).

    Database Links

    UniProt ID: P20060


    Entrez-Gene Id: 15212


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    For Research Use Only. Not For Use In Diagnostic Procedures.
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