Human Hexosaminidase A/HEXA Antibody

Catalog #: MAB62371 Datasheet / COA / SDS

Catalog #	Availability	Size / Price	Qty
MAB62371
MAB62371-SP

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

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Human Hexosaminidase A/HEXA Antibody Summary

Species Reactivity

Human

Specificity

Detects human Hexosaminidase A/HEXA in direct ELISAs.

Source

Monoclonal Mouse IgG_2B 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 either lyophilized or as a 0.2 µm filtered solution in PBS.

Endotoxin Level

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

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.

Scientific Data

Neutralization

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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.

Reconstitution Calculator

Preparation and Storage

Reconstitution

Sterile PBS to a final concentration of 0.5 mg/mL.

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

Gilbert, F. et al. (1975) Proc. Natl. Acad. Sci. USA 72:263.
Myerowitz, R. et al. (1985) Proc. Natl. Acad. Sci. USA 82:7830.
Korneluk, R.G. et al. (1986) J. Biol. Chem. 261:8407.
Mark, B.L. et al. (2003) J. Mol. Biol. 327:1093.
Mahuran, D.J. et al. (1988) J. Biol. Chem. 263:4612.
Hepbildikler, S.T. et al. (2002) J. Biol. Chem. 277:2562.
Mahuran, D.J. (1991) Biochim. Biophys. Acta 1096:87.
Mencarelli, S. et al. (2005) FEBS Lett. 579:5501.
Neufeld, E.F. (1989) J. Biol. Chem. 264:10927.
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