HEXA Antibody (E-2): sc-376735
- HEXA Antibody (E-2) is a mouse monoclonal IgG2a λ, cited in 1 publications, provided at 200 µg/ml
- raised against amino acids 377-416 mapping within an internal region of HEXA of human origin
- Anti-HEXA Antibody (E-2) is recommended for detection of HEXA of mouse, rat and human origin by WB, IP, IF and ELISA
- Anti-HEXA Antibody (E-2) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- Contact our Technical Service Department (or your local Distributor) for more information on how to receive a FREE 10 µg sample of HEXA (E-2): sc-376735.
- m-IgG2a BP-HRP and m-IgGλ BP-HRP are the preferred secondary detection reagents for HEXA Antibody (E-2) for WB applications. These reagents are now offered in bundles with HEXA Antibody (E-2) (see ordering information below).
QUICK LINKS
HEXA Antibody (E-2) is a mouse monoclonal IgG2a antibody that detects HEXA in mouse, rat, and human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). HEXA (E-2) antibody is offered in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. Hexosaminidase A (HEXA), also known as beta-Hexosaminidase A, is a crucial enzyme located in the lysosomes of cells, where HEXA plays a vital role in the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines. Proper functioning of HEXA is essential for maintaining cellular health, as HEXA deficiency leads to the accumulation of GM2 gangliosides, which is a hallmark of Tay-Sachs disease (TSD), a severe autosomal recessive lysosomal storage disorder affecting the central nervous system. TSD arises from mutations in the HEXA gene, resulting in insufficient HEXA enzyme activity and subsequent neurodegeneration due to the toxic buildup of GM2 gangliosides. Anti-HEXA antibody (E-2) is invaluable for researchers studying the biochemical pathways involved in lysosomal storage diseases and for developing potential therapeutic strategies to combat such conditions.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
HEXA Antibody (E-2) References:
- Segregation within a family of two mutant alleles for hexosaminidase A. | Kelly, TE., et al. 1976. Clin Genet. 9: 540-3. PMID: 1269177
- Hexosaminidase A deficiency is an uncommon cause of a syndrome mimicking amyotrophic lateral sclerosis. | Drory, VE., et al. 2003. Muscle Nerve. 28: 109-12. PMID: 12811781
- Production of human beta-hexosaminidase A with highly phosphorylated N-glycans by the overexpression of the Ogataea minuta MNN4 gene. | Akeboshi, H., et al. 2009. Glycobiology. 19: 1002-9. PMID: 19506294
- Tay-Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum-associated degradation. | Dersh, D., et al. 2016. Mol Biol Cell. 27: 3813-3827. PMID: 27682588
- Progranulin associates with hexosaminidase A and ameliorates GM2 ganglioside accumulation and lysosomal storage in Tay-Sachs disease. | Chen, Y., et al. 2018. J Mol Med (Berl). 96: 1359-1373. PMID: 30341570
- Functionality of a bicistronic construction containing HEXA and HEXB genes encoding β-hexosaminidase A for cell-mediated therapy of GM2 gangliosidoses. | Shaimardanova, AA., et al. 2022. Neural Regen Res. 17: 122-129. PMID: 34100447
- Hexosaminidase A (HEXA) regulates hepatic sphingolipid and lipoprotein metabolism in mice. | Montgomery, MK., et al. 2021. FASEB J. 35: e22046. PMID: 34800307
- Increasing β-hexosaminidase A activity using genetically modified mesenchymal stem cells. | Shaimardanova, AA., et al. 2024. Neural Regen Res. 19: 212-219. PMID: 37488869
- Intravenous injection of purified hexosaminidase A into a patient with Tay-Sachs disease. | Johnson, WG., et al. 1973. Birth Defects Orig Artic Ser. 9: 120-4. PMID: 4611523
- Benign HEXA mutations, C739T(R247W) and C745T(R249W), cause beta-hexosaminidase A pseudodeficiency by reducing the alpha-subunit protein levels. | Cao, Z., et al. 1997. J Biol Chem. 272: 14975-82. PMID: 9169471
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
HEXA Antibody (E-2) | sc-376735 | 200 µg/ml | $316.00 | |||
HEXA Antibody (E-2): m-IgGλ BP-HRP Bundle | sc-523071 | 200 µg Ab, 40 µg BP | $354.00 | |||
HEXA Antibody (E-2): m-IgG2a BP-HRP Bundle | sc-546236 | 200 µg Ab; 10 µg BP | $354.00 | |||
HEXA Antibody (E-2) AC | sc-376735 AC | 500 µg/ml, 25% agarose | $416.00 | |||
HEXA Antibody (E-2) HRP | sc-376735 HRP | 200 µg/ml | $316.00 | |||
HEXA Antibody (E-2) FITC | sc-376735 FITC | 200 µg/ml | $330.00 | |||
HEXA Antibody (E-2) PE | sc-376735 PE | 200 µg/ml | $343.00 | |||
HEXA Antibody (E-2) Alexa Fluor® 488 | sc-376735 AF488 | 200 µg/ml | $357.00 | |||
HEXA Antibody (E-2) Alexa Fluor® 546 | sc-376735 AF546 | 200 µg/ml | $357.00 | |||
HEXA Antibody (E-2) Alexa Fluor® 594 | sc-376735 AF594 | 200 µg/ml | $357.00 | |||
HEXA Antibody (E-2) Alexa Fluor® 647 | sc-376735 AF647 | 200 µg/ml | $357.00 | |||
HEXA Antibody (E-2) Alexa Fluor® 680 | sc-376735 AF680 | 200 µg/ml | $357.00 | |||
HEXA Antibody (E-2) Alexa Fluor® 790 | sc-376735 AF790 | 200 µg/ml | $357.00 |