Human Hexosaminidase A/HEXA Antibody

Catalog # Availability Size / Price Qty
MAB6237
MAB6237-SP
Detection of Human Hexosaminidase A/HEXA by Western Blot.
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Human Hexosaminidase A/HEXA Antibody Summary

Species Reactivity
Human
Specificity
Detects human Hexosaminidase A/HEXA in direct ELISAs and Western blots.
Source
Monoclonal Mouse IgG2B Clone # 714729
Purification
Protein A or G purified from hybridoma culture supernatant
Immunogen
S. frugiperda insect ovarian cell line Sf 21-derived recombinant human Hexosaminidase A/HEXA
Met1-Thr529
Accession # P06865
Formulation
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.

Applications

Recommended Concentration
Sample
Western Blot
2 µg/mL
See below
Immunohistochemistry
8-25 µg/mL
See below

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Scientific Data

Western Blot Detection of Human Hexosaminidase A/HEXA antibody by Western Blot. View Larger

Detection of Human Hexosaminidase A/HEXA by Western Blot. Western blot shows lysates of HepG2 human hepatocellular carcinoma cell line and human liver tissue. PVDF membrane was probed with 2 µg/mL of Mouse Anti-Human Hexosaminidase A/HEXA Monoclonal Antibody (Catalog # MAB6237) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog # HAF007). A specific band was detected for Hexosaminidase A/HEXA at approximately 60 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Immunohistochemistry Hexosaminidase A/HEXA antibody in Human Brain by Immunohistochemistry (IHC-P). View Larger

Hexosaminidase A/HEXA in Human Brain. Hexosaminidase A/HEXA was detected in immersion fixed paraffin-embedded sections of human brain (hypothalamus) using Mouse Anti-Human Hexosaminidase A/HEXA Monoclonal Antibody (Catalog # MAB6237) at 15 µg/mL overnight at 4 °C. Before incubation with the primary antibody, tissue was subjected to heat-induced epitope retrieval using Antigen Retrieval Reagent-Basic (Catalog # CTS013). Tissue was stained using the Anti-Mouse HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # CTS002) and counterstained with hematoxylin (blue). Specific staining was localized to the cytoplasm and lysosomes in neuronal cell bodies. View our protocol for Chromogenic IHC Staining of Paraffin-embedded Tissue Sections.

Reconstitution Calculator

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

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Preparation and Storage

Reconstitution
Sterile PBS to a final concentration of 0.5 mg/mL.
Reconstitution Buffer Available
Reconstitution Buffer 1 (PBS)
Catalog #
Availability
Size / Price
Qty
RB01
Shipping
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
Stability & 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.

Background: Hexosaminidase A/HEXA

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.

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. 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.
Entrez Gene IDs
3073 (Human)
Alternate Names
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

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Isotype Controls

Reconstitution Buffers

Secondary Antibodies

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Human Hexosaminidase A/HEXA Antibody
By Anonymous on 01/30/2023
Application: Non-specific staining Sample Tested: HEK293T human embryonic kidney cell line Species: Human