Hexosaminidase A/HEXA Antibody (714712) [Unconjugated] Summary
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. |
Source |
N/A |
Isotype |
IgG2b |
Clonality |
Monoclonal |
Host |
Mouse |
Gene |
HEXA |
Purity Statement |
Protein A or G purified from hybridoma culture supernatant |
Endotoxin Note |
<0.10 EU per 1 μg of the antibody by the LAL method. |
Innovator's Reward |
Test in a species/application not listed above to receive a full credit towards a future purchase. |
Applications/Dilutions
Packaging, Storage & Formulations
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.
|
Buffer |
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. |
Preservative |
No Preservative |
Concentration |
LYOPH |
Reconstitution Instructions |
Sterile PBS to a final concentration of 0.5 mg/mL. |
Notes
This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.
Alternate Names for Hexosaminidase A/HEXA Antibody (714712) [Unconjugated]
Background
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.
- 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.
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are
guaranteed for 1 year from date of receipt.
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