- Tel: 858.663.9055
-
Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
Hexosaminidase B Antibody HEXB/7762 / Lysosomal Beta-Hexosaminidase Antibody [clone HEXB/7762] (V5089)
Email: info@nsjbio.com
| Catalog No | Formulation | Size | Price (USD) | ||
|---|---|---|---|---|---|
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V5089-100UG | 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced), 0.05% sodium azide | 100 ug | 559 | |
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V5089-20UG | 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced), 0.05% sodium azide | 20 ug | 259 | |
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V5089SAF-100UG | 1 mg/ml in 1X PBS; BSA free, sodium azide free | 100 ug | 559 |
| Availability | 1-3 business days |
| Species Reactivity | Human |
| Format | Purified |
| Host | Mouse |
| Clonality | Monoclonal (mouse origin) |
| Isotype | Mouse IgG2, kappa |
| Clone Name | HEXB/7762 |
| Purity | Protein A/G affinity |
| UniProt | P07686 |
| Localization | Lysosome |
| Applications | Immunohistochemistry (FFPE) : 1-2ug/ml for 30 min at RT |
| Limitations | This Hexosaminidase B Antibody HEXB/7762 / Lysosomal Beta-Hexosaminidase Antibody is available for research use only. |
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Hexosaminidase B Antibody HEXB/7762 / Lysosomal Beta-Hexosaminidase Antibody recognizes the beta subunit of beta-hexosaminidase, encoded by the HEXB gene. HEXB is synthesized as a precursor protein that undergoes processing within the endosomal-lysosomal system to form a mature lysosomal enzyme subunit. The beta subunit can associate with another beta subunit to form beta-hexosaminidase B (Hex B), or with the alpha subunit encoded by HEXA to form beta-hexosaminidase A (Hex A).
Hex A and Hex B are lysosomal glycosidases that participate in the degradation of glycosphingolipids and other glycoconjugates. Hex A, composed of alpha and beta subunits, works with the GM2 activator protein to degrade GM2 ganglioside. Hex B is a beta-beta homodimer with overlapping but distinct substrate specificity. Through its participation in both enzyme complexes, the HEXB beta subunit is an important component of normal lysosomal catabolism.
Pathogenic variants in HEXB cause Sandhoff disease, a GM2 gangliosidosis characterized by deficient activity of both Hex A and Hex B. Loss of beta-hexosaminidase function results in progressive accumulation of GM2 ganglioside and related substrates, with particularly severe consequences in the nervous system. HEXB is therefore widely studied in lysosomal storage disease, sphingolipid metabolism, neurodegeneration and mechanisms governing lysosomal function.
Clone HEXB/7762 is a mouse monoclonal antibody generated against recombinant human HEXB protein. NSJ Bioreagents provides this Hexosaminidase B Antibody for research into beta-hexosaminidase expression, lysosomal biology and GM2 ganglioside metabolism. A Lysosomal Beta-Hexosaminidase Antibody can support investigation of the HEXB-containing enzyme complexes and their roles in cellular glycoconjugate degradation.
Explore our Neuroscience Antibodies page for additional reagents targeting proteins involved in neuronal function, neurodegeneration and neurological disease research.
Optimal dilution of the Hexosaminidase B Antibody HEXB/7762 / Lysosomal Beta-Hexosaminidase Antibody should be determined by the researcher.
A recombinant fragment of the human protein was used as the immunogen for the Hexosaminidase B antibody.
Aliquot the Hexosaminidase B antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
Hexosaminidase B antibody, HEXB antibody, beta-hexosaminidase beta subunit antibody, beta-hexosaminidase subunit beta antibody, Hex B antibody
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