Recombinant Human Hexosaminidase A/HEXA Protein, CF
R&D Systems, part of Bio-Techne | Catalog # 6237-GH
Key Product Details
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Product Specifications
Source
Spodoptera frugiperda, Sf 21 (baculovirus)-derived human Hexosaminidase A/HEXA protein
Met1-Thr529, with a C-terminal 6-His tag
Met1-Thr529, with a C-terminal 6-His tag
Purity
>95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining.
Endotoxin Level
<1.0 EU per 1 μg of the protein by the LAL method.
N-terminal Sequence Analysis
Leu23
Predicted Molecular Mass
59 kDa
SDS-PAGE
57-61 kDa, reducing conditions
Activity
Measured by its ability to hydrolyze 4-methylumbelliferyl-N-acetyl-beta -D-glucosaminide (4-MU-GlcNAc)
The specific activity is >1,250 pmol/min/μg, as measured under the described conditions.
The specific activity is >1,250 pmol/min/μg, as measured under the described conditions.
Formulation, Preparation and Storage
6237-GH
| Formulation | Supplied as a 0.2 μm filtered solution in Tris and NaCl. |
| Shipping | The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below. |
| Stability & Storage | Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
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Background: Hexosaminidase A/HEXA
(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. Our recombinant HEXA is presumably isoform Hex S, because only alpha subunit was expressed. 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.
Alternate Names
HEXA, TSD
Entrez Gene IDs
3073 (Human)
Gene Symbol
HEXA
UniProt
Additional Hexosaminidase A/HEXA Products
Product Documents for Recombinant Human Hexosaminidase A/HEXA Protein, CF
Product Specific Notices for Recombinant Human Hexosaminidase A/HEXA Protein, CF
For research use only
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