Recombinant Human Hexosaminidase A/HEXA Protein, CF

Images

 
There are currently no images for Hexosaminidase A/HEXA (6237-GH).

Every product we sell is backed by Novus' 100% Guarantee. If you have used this product, please submit your images and reviews to earn reward points.

Product Details

Summary
Reactivity HuSpecies Glossary
Applications Enzyme Activity
Format
Carrier-Free

Order Details

Recombinant Human Hexosaminidase A/HEXA Protein, CF Summary

Details of Functionality
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.
Source
Spodoptera frugiperda, Sf 21 (baculovirus)-derived human Hexosaminidase A/HEXA protein
Met1-Thr529, with a C-terminal 6-His tag
Accession #
N-terminal Sequence
Leu23
Protein/Peptide Type
Recombinant Enzymes
Gene
HEXA
Purity
>95%, by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie® Blue stain at 5 μg per lane
Endotoxin Note
<1.0 EU per 1 μg of the protein by the LAL method.

Applications/Dilutions

Theoretical MW
59 kDa.
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.
SDS-PAGE
57-61 kDa, reducing conditions
Publications
Read Publication using
6237-GH in the following applications:

Packaging, Storage & Formulations

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.
Buffer
Supplied as a 0.2 μm filtered solution in Tris and NaCl.
Purity
>95%, by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie® Blue stain at 5 μg per lane
Assay Procedure
  • Assay Buffer: 100 mM Sodium Citrate, 250 mM NaCl, pH 4.5
  • Recombinant Human Hexosaminidase A/HEXA (rhHEXA) (Catalog # 6237-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 rhHEXA to 5 ng/μL in Assay Buffer.
  2. Dilute Substrate to 800 μM in Assay Buffer.
  3. Load into a plate 50 μL of 5 ng/μL rhHEXA, and start the reaction by adding 50 μL of 800 μM Substrate. For Substrate Blanks, 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:
  • rhHEXA: 0.250 μg
  • Substrate: 400 μM

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for Recombinant Human Hexosaminidase A/HEXA Protein, CF

  • beta-hexosaminidase subunit alpha
  • Beta-N-acetylhexosaminidase subunit alpha
  • EC 3.2.1
  • EC 3.2.1.52
  • HEXA
  • hexosaminidase A (alpha polypeptide)
  • Hexosaminidase A
  • Hexosaminidase subunit A
  • MGC99608
  • N-acetyl-beta-glucosaminidase subunit alpha
  • TSD

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. 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.
  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. Hepbildikler, S.T. et al. (2002) J. Biol. Chem. 277:2562.
  7. Mahuran, D.J. (1991) Biochim. Biophys. Acta 1096:87.
  8. Mencarelli, S. et al. (2005) FEBS Lett. 579:5501.
  9. Neufeld, E.F. (1989) J. Biol. Chem. 264:10927.
  10. Ohno, K. et al. (2008) Mol. Genet. Metab. 94:462.

Customers Who Viewed This Item Also Viewed...

H00003087-M02
Species: Hu
Applications: WB, ELISA, ICC/IF
8907-GH
Species: Hu
NBP1-88927
Species: Hu
Applications: WB, ICC/IF, IHC, IHC-P
NBP1-43435
Species: Hu, Mu
Applications: WB, Flow
H00728695-B01P
Species: Hu
Applications: WB, ELISA, ICC/IF, IHC-P
NBP1-81838
Species: Hu, Mu
Applications: WB, IHC, IHC-P
NBP1-83574
Species: Hu
Applications: WB, ICC/IF, IHC, IHC-P
AF1067
Species: Hu, Mu, Rt
Applications: WB, ELISA, IHC, Neut
NB200-541
Species: Mu
Applications: WB, ICC/IF, IHC, IHC-Fr, IP
MAB25031
Species: Hu
Applications: WB, IHC, IP
NBP2-02043
Species: Hu
Applications: WB, Flow, ICC/IF, IHC, IHC-P
AF1067
Species: Hu, Mu, Rt
Applications: WB, ELISA, IHC, Neut
NBP2-67569
Species: Hu, Mu, Rt
Applications: WB, Flow, ICC/IF, IHC, IHC-P
NBP1-84854
Species: Hu, Mu, Rt
Applications: WB, ICC/IF, IHC, IHC-P, KD
AF2009
Species: Hu
Applications: WB, IHC
6237-GH
Species: Hu
Applications: Enzyme Activity

Publications for Hexosaminidase A/HEXA (6237-GH)(1)

We have publications tested in 1 confirmed species: N/A.

We have publications tested in 1 application: Bioassay.


Filter By Application
Bioassay
(1)
All Applications
Filter By Species
N/A
(1)
All Species

Reviews for Hexosaminidase A/HEXA (6237-GH) (0)

There are no reviews for Hexosaminidase A/HEXA (6237-GH). By submitting a review you will receive an Amazon e-Gift Card or Novus Product Discount.
  • Review with no image -- $10/€7/£6/$10 CAD/¥70 Yuan/¥1110 Yen
  • Review with an image -- $25/€18/£15/$25 CAD/¥150 Yuan/¥2500 Yen
  • 1st to review with an image -- $50/€35/£30/$50 CAD/¥300 Yuan/¥5000 Yen

FAQs for Hexosaminidase A/HEXA (6237-GH) (0)

There are no specific FAQs related to this product. Read our general customer & technical service FAQs.

Additional Array Products

Bioinformatics Tool for Hexosaminidase A/HEXA (6237-GH)

Discover related pathways, diseases and genes to Hexosaminidase A/HEXA (6237-GH). Need help? Read the Bioinformatics Tool Guide for instructions on using this tool.
Visit Tool

Diseases for Hexosaminidase A/HEXA (6237-GH)

Discover more about diseases related to Hexosaminidase A/HEXA (6237-GH).
 

Pathways for Hexosaminidase A/HEXA (6237-GH)

View related products by pathway.

PTMs for Hexosaminidase A/HEXA (6237-GH)

Learn more about PTMs related to Hexosaminidase A/HEXA (6237-GH).

Blogs on Hexosaminidase A/HEXA

There are no specific blogs for Hexosaminidase A/HEXA, but you can read our latest blog posts.

Customers Who Bought This Also Bought

CD20 Antibody (AISB12)
NBP1-43435-0.1mg

Contact Information

Product PDFs

Calculators

Concentration Calculator

The concentration calculator allows you to quickly calculate the volume, mass or concentration of your vial. Simply enter your mass, volume, or concentration values for your reagent and the calculator will determine the rest.

=
÷

Review this Product

Be the first to review our Recombinant Human Hexosaminidase A/HEXA Protein, CF and receive a gift card or discount.

Bioinformatics

Gene Symbol HEXA
Entrez
Uniprot