Detects human Hexosaminidase A/HEXA in direct ELISAs.
Monoclonal Mouse IgG2B Clone # 714712
Protein A or G purified from hybridoma culture supernatant
S. frugiperda insect ovarian cell line Sf 21-derived recombinant human Hexosaminidase A/HEXA Met1-Thr529 Accession # P06865
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.
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.
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
Sterile PBS to a final concentration of 0.5 mg/mL.
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.
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.
Have you used Human Hexosaminidase A/HEXA Antibody?
Submit a review and receive a $25US/€18/£15/$25CAN amazon gift card if you include an image - $10US/€7/£6/$10CAN Amazon card for reviews without an image. Limited to verified customers in USA, Canada and Europe.