Recombinant Human Hexosaminidase B/HEXB Protein, CF

R&D Systems, part of Bio-Techne | Catalog # 8907-GH

R&D Systems, part of Bio-Techne
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8907-GH-020
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Key Product Details

Source

CHO

Accession #

P07686

Conjugate

Unconjugated

Applications

Enzyme Activity

View all Hexosaminidase B/HEXB Proteins and Enzymes »

Product Specifications

Source

Chinese Hamster Ovary cell line, CHO-derived human Hexosaminidase B/HEXB protein
Ala43-Met556, with C-terminal 6-His

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

Ala43

Predicted Molecular Mass

60 kDa

SDS-PAGE

59-70 kDa, reducing conditions

Activity

Measured by its ability to hydrolyze 4-methylumbelliferyl-N-acetyl-beta -D-glucosaminide (4-MU-GlcNAc)
The specific activity is >6,000 pmol/min/μg, as measured under the described conditions.

Formulation, Preparation and Storage

8907-GH
Formulation Supplied as a 0.2 μm filtered solution in Tris, NaCl and Glycerol.
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.
  • 6 months from date of receipt, -20 to -70 °C as supplied.
  • 3 months, -20 to -70 °C under sterile conditions after opening.

Background: Hexosaminidase B/HEXB

Beta-hexosaminidases are enzymes involved in the hydrolysis of terminal N-acetyl-D-hexosamine residues in GM2 gangliosides and globo sphingolipids in lysosomes in the brain and other tissues (1-4). These enzymes are composed of alpha and/or beta subunits, which are coded by HEXA and HEXB genes, respectively. Different combination of alpha and beta subunits gives rise to beta-hexosaminidase isoform A, B and S (Hex A, B and S) (5), which have the composition of alpha beta, beta beta and alpha alpha, respectively. The recombinant HEXB is presumably in the B isoform and is highly active on 4-methylumbelliferyl-N-acetyl-beta -D-glucosaminide. Mutations in HEXA and HEXB genes cause lysosomal lipid storage disorders, such as Tay-Sachs disease, manifested by harmful accumulation of ganglioside GM2 in tissues and nerve cells in the brain (6-9). Also, mutation of HEXB results in Sandhoff disease manifested by the accumulation of both ganglioside GM2 and globoside in tissues and nerve cells in the brain (10, 11).

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. Mahuran, D.J.1991, Biochim. Biophys. Acta. 1096:87.
  7. Mencarelli, S. et al.2005, FEBS Lett. 579:5501.
  8. Neufeld, E.F.1989, J. Biol. Chem. 264:10927.
  9. Ohno, K. et al.2008, Mol. Genet. Metab. 94:462.
  10. Maier T., et al. 2003 J. Mol. Biol. 328:669.
  11. Gomez-Lira M., et al. 1995 Hum. Genet. 96:417.

Alternate Names

ENC-1AS, HCC-7, HEL-248, HEXB

Entrez Gene IDs

3074 (Human); 15212 (Mouse); 294673 (Rat)

Gene Symbol

HEXB

UniProt

P07686

Additional Hexosaminidase B/HEXB Products

Product Documents for Recombinant Human Hexosaminidase B/HEXB Protein, CF

Product Datasheets

Certificate of Analysis

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Product Specific Notices for Recombinant Human Hexosaminidase B/HEXB Protein, CF

For research use only

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