GM2-AP Antibody (E-7): sc-514437
- GM2-AP Antibody (E-7) is a mouse monoclonal IgG1 κ GM2-AP antibody, cited in 1 publications, provided at 200 µg/ml
- raised against amino acids 1-193 representing full length GM2-AP of human origin
- GM2-AP Antibody (E-7) is recommended for detection of GM2-AP of human origin by WB, IP, IF and ELISA
- Anti-GM2-AP Antibody (E-7) 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 GM2-AP (E-7): sc-514437.
- m-IgG Fc BP-HRP is the preferred secondary detection reagent for GM2-AP Antibody (E-7) for WB applications. This reagent is now offered in a bundle with GM2-AP Antibody (E-7) (see ordering information below).
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GM2-AP Antibody (E-7) is a mouse monoclonal IgG1 kappa light chain antibody that detects GM2-AP of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). GM2-AP (E-7) antibody is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. GM2-AP, also known as GM2 ganglioside activator, GM2A, or SAP-3 (sphingolipid activator protein 3), is a 193 amino acid protein primarily localized to the lysosome, where GM2-AP plays a crucial role in lipid metabolism. As a small glycolipid transport protein, GM2-AP acts as a ganglioside-specific cofactor that enhances beta-hexosaminidase A (HEXA) activity, facilitating GA2 glycolipid and GM2 ganglioside breakdown. This function is vital for degrading proteins containing terminal N-acetyl hexosamines, and any disruption in this process can lead to severe metabolic disorders. Notably, mutations in the gene encoding GM2-AP are responsible for GM2-gangliosidosis type AB (GM2GAB), also known as Tay-Sachs disease AB variant, an autosomal recessive condition characterized by GM2 ganglioside accumulation despite hexosaminidase A and B presence, underscoring GM2-AP′s importance in maintaining cellular lipid homeostasis.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
GM2-AP Antibody (E-7) References:
- Structure of the GM2A gene: identification of an exon 2 nonsense mutation and a naturally occurring transcript with an in-frame deletion of exon 2. | Chen, B., et al. 1999. Am J Hum Genet. 65: 77-87. PMID: 10364519
- Crystal structure of human GM2-activator protein with a novel beta-cup topology. | Wright, CS., et al. 2000. J Mol Biol. 304: 411-22. PMID: 11090283
- Identification of a processed pseudogene related to the functional gene encoding the GM2 activator protein: localization of the pseudogene to human chromosome 3 and the functional gene to human chromosome 5. | Xie, B., et al. 1992. Genomics. 14: 796-8. PMID: 1427911
- Evidence for two cDNA clones encoding human GM2-activator protein. | Nagarajan, S., et al. 1992. Biochem J. 282 (Pt 3): 807-13. PMID: 1554364
- Characterization of full-length cDNAs and the gene coding for the human GM2 activator protein. | Klima, H., et al. 1991. FEBS Lett. 289: 260-4. PMID: 1915857
- A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB. | Schröder, M., et al. 1991. FEBS Lett. 290: 1-3. PMID: 1915858
- Isolation and expression of a full-length cDNA encoding the human GM2 activator protein. | Xie, B., et al. 1991. Biochem Biophys Res Commun. 177: 1217-23. PMID: 2059210
- Isolation of a cDNA encoding the human GM2 activator protein. | Schröder, M., et al. 1989. FEBS Lett. 251: 197-200. PMID: 2753159
- Molecular genetics of GM2-gangliosidosis AB variant: a novel mutation and expression in BHK cells. | Schröder, M., et al. 1993. Hum Genet. 92: 437-40. PMID: 8244332
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
GM2-AP Antibody (E-7) | sc-514437 | 200 µg/ml | $316.00 | |||
GM2-AP Antibody (E-7): m-IgG Fc BP-HRP Bundle | sc-526369 | 200 µg Ab; 10 µg BP | $354.00 | |||
GM2-AP Antibody (E-7) AC | sc-514437 AC | 500 µg/ml, 25% agarose | $416.00 | |||
GM2-AP Antibody (E-7) HRP | sc-514437 HRP | 200 µg/ml | $316.00 | |||
GM2-AP Antibody (E-7) FITC | sc-514437 FITC | 200 µg/ml | $330.00 | |||
GM2-AP Antibody (E-7) PE | sc-514437 PE | 200 µg/ml | $343.00 | |||
GM2-AP Antibody (E-7) Alexa Fluor® 488 | sc-514437 AF488 | 200 µg/ml | $357.00 | |||
GM2-AP Antibody (E-7) Alexa Fluor® 546 | sc-514437 AF546 | 200 µg/ml | $357.00 | |||
GM2-AP Antibody (E-7) Alexa Fluor® 594 | sc-514437 AF594 | 200 µg/ml | $357.00 | |||
GM2-AP Antibody (E-7) Alexa Fluor® 647 | sc-514437 AF647 | 200 µg/ml | $357.00 | |||
GM2-AP Antibody (E-7) Alexa Fluor® 680 | sc-514437 AF680 | 200 µg/ml | $357.00 | |||
GM2-AP Antibody (E-7) Alexa Fluor® 790 | sc-514437 AF790 | 200 µg/ml | $357.00 |