Human Hexosaminidase A/HEXA Antibody

R&D Systems, part of Bio-Techne | Catalog # MAB62371

R&D Systems, part of Bio-Techne
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MAB62371
MAB62371-SP
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Key Product Details

Species Reactivity

Human

Applications

Neutralization

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG2B Clone # 714712

View all Hexosaminidase A/HEXA Antibodies »

Product Specifications

Immunogen

S. frugiperda insect ovarian cell line Sf 21-derived recombinant human Hexosaminidase A/HEXA
Met1-Thr529
Accession # P06865

Specificity

Detects human Hexosaminidase A/HEXA in direct ELISAs.

Clonality

Monoclonal

Host

Mouse

Isotype

IgG2B

Endotoxin Level

<0.10 EU per 1 μg of the antibody by the LAL method.

Scientific Data Images for Human Hexosaminidase A/HEXA Antibody

Neutralization of Human Hexosaminidase A Activity by Human Hexosaminidase A Antibody.

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, 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.

Applications for Human Hexosaminidase A/HEXA Antibody

Application
Recommended Usage

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.

Formulation, Preparation, and Storage

Purification

Protein A or G purified from hybridoma culture supernatant

Reconstitution

Sterile PBS to a final concentration of 0.5 mg/mL. For liquid material, refer to CoA for concentration.

Reconstitution Buffer Available:
Size / Price
Qty

Formulation

Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.

Shipping

Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.

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.

Alternate Names

HEXA, TSD

Entrez Gene IDs

3073 (Human)

Gene Symbol

HEXA

UniProt

P06865

Additional Hexosaminidase A/HEXA Products

Product Documents for Human Hexosaminidase A/HEXA Antibody

Product Datasheets

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

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Product Specific Notices for Human Hexosaminidase A/HEXA Antibody

For research use only

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