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HEXA Antibody
Purified Mouse Monoclonal Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, FC, E |
|---|---|
| Primary Accession | P06865 |
| Reactivity | Human |
| Host | Mouse |
| Clonality | Monoclonal |
| Clone Names | 3F10 |
| Isotype | IgG2b |
| Calculated MW | 60.7kDa |
| Description | This gene encodes the alpha subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I). |
| Immunogen | Purified recombinant fragment of human HEXA expressed in E. Coli. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide |
| Gene ID | 3073 |
|---|---|
| Other Names | Beta-hexosaminidase subunit alpha, 3.2.1.52, Beta-N-acetylhexosaminidase subunit alpha, Hexosaminidase subunit A, N-acetyl-beta-glucosaminidase subunit alpha, HEXA |
| Dilution | E~~1/10000 WB~~1/500 - 1/2000 FC~~1/200 - 1/400 |
| Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Precautions | HEXA Antibody is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | HEXA (HGNC:4878) |
|---|---|
| Function | Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides (PubMed:11707436, PubMed:8123671, PubMed:8672428, PubMed:9694901). The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6- sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2 (PubMed:11707436). The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide (PubMed:11707436). Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A (PubMed:8123671, PubMed:8672428, PubMed:9694901). |
| Cellular Location | Lysosome. |
Research Areas
Citations (0)
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info@abcepta.com, and receive a free "I Love Antibodies" mug.
info@abcepta.com, and receive a free "I Love Antibodies" mug.
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
References
Clin Biochem. 2009 Jul;42(10-11):1187-9. Pediatr Res. 2010 Feb;67(2):217-20.
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$ 385.00
Cat# AO1693a
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