Human Hexosaminidase A/HEXA Antibody

  
  • Species Reactivity
    Human
  • Specificity
    Detects human Hexosaminidase A/HEXA in direct ELISAs.
  • Source
    Monoclonal Mouse IgG2B Clone # 714712
  • Purification
    Protein A or G purified from hybridoma culture supernatant
  • Immunogen
    S. frugiperda insect ovarian cell line Sf 21-derived recombinant human Hexosaminidase A/HEXA
    Met1-Thr529
    Accession # P06865
  • Formulation
    Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.
  • Label
    Unconjugated
Applications
  •  
    Recommended
    Concentration
    Sample
  • Neutralization
    Measured by its ability to neutralize Human Hexosaminidase A (2.0 μg/mL,Catalog # 6237-GH) cleavage of the fluorogenic peptide substrate 4-Methylumbelliferyl-N-Acetyl-beta -D-glucosaminide (400 µM). The Neutralization Dose (ND50) is typically 3.0 µg/mL.
Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.
Data Examples
Neutralization of Human Hexosaminidase A Activity by Human Hexosaminidase A Antibody. The cleavage of 4-Methylumbelliferyl-N-Acetyl-beta -D-glucosaminide (400 μM) by Human Hexosaminidase A (2.0 μg/mL,Catalog # 6237-GH) is measured after it has been preincubated with increasing concentrations of Mouse Anti-Human Hexosaminidase A Monoclonal Antibody (Catalog # MAB62371). The ND50 is typically 3.0 μg/mL.
Preparation and Storage
  • Reconstitution
    Sterile PBS to a final concentration of 0.5 mg/mL.
  • Shipping
    The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
  • Stability & Storage
    Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
    • 12 months from date of receipt, -20 to -70 °C as supplied.
    • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
    • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
Background: Hexosaminidase A/HEXA
beta -hexosaminidases are enzymes involved in the hydrolysis of terminal N-acetyl-D-hexosamine residues in GM2 gangliosides and globo-sphingolipids in lysosomes (1‑4). The enzymes are composed of two alpha and/or beta subunits, which are coded by HEXA and HEXB genes, respectively. Different association of the alpha and beta subunits gives rise to beta ‑hexosaminidase isoforms A, B and S (Hex A, B and S) (5), which have the composition of alpha beta, beta beta and alpha alpha, respectively. Hex S is suggested to releases non‑reducing end N-acetylgalactosamine residues from dermatan sulfate, chondroitin sulfate and sulfated glycolipid SM2 (6). Recombinant HEXA is also highly active on 4-methylumbelliferyl-N-acetyl-beta -D-glucosaminide (6). Mutations in HEXA and HEXB genes cause lysosomal lipid storage disorders. Specifically, mutations of HEXA cause Tay-Sachs disease, manifested by the harmful accumulation of ganglioside GM2 in tissues and nerve cells in the brain (7‑10). Children with this disease usually die by age 4.
  • References:
    1. Gilbert, F. et al. (1975) Proc. Natl. Acad. Sci. USA 72:263.
    2. Myerowitz, R. et al. (1985) Proc. Natl. Acad. Sci. USA 82:7830.
    3. Korneluk, R.G. et al. (1986) J. Biol. Chem. 261:8407.
    4. Mark, B.L. et al. (2003) J. Mol. Biol. 327:1093.
    5. Mahuran, D.J. et al. (1988) J. Biol. Chem. 263:4612.
    6. Hepbildikler, S.T. et al. (2002) J. Biol. Chem. 277:2562.
    7. Mahuran, D.J. (1991) Biochim. Biophys. Acta 1096:87.
    8. Mencarelli, S. et al. (2005) FEBS Lett. 579:5501.
    9. Neufeld, E.F. (1989) J. Biol. Chem. 264:10927.
    10. Ohno, K. et al. (2008) Mol. Genet. Metab. 94:462.
  • Entrez Gene IDs:
    3073 (Human)
  • Alternate Names:
    beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; EC 3.2.1; EC 3.2.1.52; HEXA; hexosaminidase A (alpha polypeptide); Hexosaminidase A; Hexosaminidase subunit A; MGC99608; N-acetyl-beta-glucosaminidase subunit alpha; TSD
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