Recombinant Human Hexosaminidase B/HEXB Protein, CF

R&D Systems | Catalog # 8907-GH

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Key Product Details

  • R&D Systems CHO-derived Recombinant Human Hexosaminidase B/HEXB Protein (8907-GH)
  • Quality control testing to verify active proteins with lot specific assays by in-house scientists
  • All R&D Systems proteins are covered with a 100% guarantee

Source

CHO

Accession Number

P07686

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

Certificate of Analysis

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

For research use only

Citations for Recombinant Human Hexosaminidase B/HEXB Protein, CF

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Protocols

View specific protocols for Recombinant Human Hexosaminidase B/HEXB Protein, CF (8907-GH):

Materials
  • Assay Buffer:  0.1 M MES, pH 5.5
  • Recombinant Human Hexosaminidase B/HEXB (rhHEXB) (Catalog # 8907-GH)
  • Substrate: 4-Methylumbelliferyl-N-Acetyl-beta -D-glucosaminide (4-MU-GlcNAc) (Sigma, Catalog # M2133), 50 mM stock in DMSO
  • F16 Black Maxisorp Plate (Nunc, Catalog # 475515)
  • Fluorescent Plate Reader (Model: SpectraMax Gemini EM by Molecular Devices) or equivalent
  1. Dilute rhHEXB to 2 ng/μL in Assay Buffer.
  2. Dilute Substrate to 800 μM in Assay Buffer.
  3. Load into a plate 50 μL of 2 ng/μL rhHEXB, and start the reaction by adding 50 μL of 800 μM Substrate. For Substrate Blank, load 50 μL of Assay Buffer and 50 μL of 800 μM Substrate.
  4. Read plate at excitation and emission wavelengths of 365 nm and 445 nm, respectively, in kinetic mode for 5 minutes.
  5. Calculate specific activity:

     Specific Activity (pmol/min/µg) =

 Adjusted Vmax* (RFU/min) x Conversion Factor** (pmol/RFU)
amount of enzyme (µg)

      *Adjusted for Substrate Blank
      **Derived using calibration standard 4-Methylumbelliferone (4-MU) (Sigma, Catalog # M1381) Per Well:
  • rhHEXB: 0.1 μg
  • Substrate: 400 μM

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