Human Hexosaminidase A/HEXA Antibody

R&D Systems, part of Bio-Techne | Catalog # MAB6237

R&D Systems, part of Bio-Techne
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MAB6237
MAB6237-SP
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Key Product Details

Species Reactivity

Human

Applications

Immunohistochemistry, Western Blot

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG2B Clone # 714729

View all Hexosaminidase A/HEXA Antibodies »

Product Specifications

Immunogen

S. frugiperda insect ovarian cell line Sf 21-derived recombinant human Hexosaminidase A/HEXA
Met1-Thr529
Accession # P06865

Specificity

Detects human Hexosaminidase A/HEXA in direct ELISAs and Western blots.

Clonality

Monoclonal

Host

Mouse

Isotype

IgG2B

Scientific Data Images for Human Hexosaminidase A/HEXA Antibody

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

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

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.

Applications for Human Hexosaminidase A/HEXA Antibody

Application
Recommended Usage

Immunohistochemistry

8-25 µg/mL
Sample: Immersion fixed paraffin-embedded sections of human brain (hypothalamus)

Western Blot

2 µg/mL
Sample: HepG2 human hepatocellular carcinoma cell line and human liver tissue

Reviewed Applications

Read 1 review rated 3 using MAB6237 in the following applications:

Formulation, Preparation, and Storage

Purification

Protein A or G purified from hybridoma culture supernatant

Reconstitution

Sterile PBS to a final concentration of 0.5 mg/mL. For liquid material, refer to CoA for concentration.

Reconstitution Buffer Available:
Size / Price
Qty

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.

Shipping

Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.

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.

Alternate Names

HEXA, TSD

Entrez Gene IDs

3073 (Human)

Gene Symbol

HEXA

UniProt

P06865

Additional Hexosaminidase A/HEXA Products

Product Documents for Human Hexosaminidase A/HEXA Antibody

Product Datasheets

Certificate of Analysis

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Product Specific Notices for Human Hexosaminidase A/HEXA Antibody

For research use only

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