- Tel: 858.663.9055
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Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
HEXB Antibody / Beta-Hexosaminidase Beta Subunit Antibody (R31624)
Email: info@nsjbio.com
| Catalog No | Formulation | Size | Price (USD) | ||
|---|---|---|---|---|---|
|
R31624 | 0.5mg/ml if reconstituted with 0.2ml sterile DI water | 100 ug | 449 |
| Availability | 1-3 business days |
| Species Reactivity | Human |
| Format | Antigen affinity purified |
| Host | Rabbit |
| Clonality | Polyclonal (rabbit origin) |
| Isotype | Rabbit IgG |
| Purity | Antigen affinity |
| Buffer | Lyophilized from 1X PBS with 2.5% BSA and 0.025% sodium azide |
| Gene ID | 3074 |
| Applications | Western Blot : 0.5-1ug/ml IHC (FFPE) : 0.5-1ug/ml |
| Limitations | This HEXB Antibody / Beta-Hexosaminidase Beta Subunit Antibody is available for research use only. |
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HEXB Antibody / Beta-Hexosaminidase Beta Subunit Antibody recognizes the beta subunit of beta-hexosaminidase, encoded by the HEXB gene. HEXB is synthesized as a precursor that is transported through the secretory pathway and processed within the endosomal-lysosomal system. The mature beta subunit contributes to two lysosomal beta-hexosaminidase enzymes with different subunit compositions and substrate specificities.
HEXB beta subunits associate as homodimers to form beta-hexosaminidase B (Hex B) and combine with alpha subunits encoded by HEXA to form beta-hexosaminidase A (Hex A). These lysosomal glycosidases participate in the degradation of glycosphingolipids, glycoproteins and other glycoconjugates. Hex A additionally works with GM2 activator protein in the degradation of GM2 ganglioside, connecting HEXB directly to lysosomal sphingolipid metabolism.
Loss-of-function variants in HEXB cause Sandhoff disease, a lysosomal storage disorder within the GM2 gangliosidosis group. Because the beta subunit participates in both Hex A and Hex B, HEXB deficiency compromises the activity of both enzymes and leads to accumulation of GM2 ganglioside and other undegraded substrates. Progressive substrate accumulation particularly affects the nervous system, making HEXB relevant to research involving lysosomal dysfunction, lipid metabolism and neurodegeneration.
This rabbit polyclonal antibody was generated against a recombinant fragment of human HEXB and is antigen affinity purified. NSJ Bioreagents provides this HEXB Antibody for research into beta-hexosaminidase expression, lysosomal metabolism and the molecular biology of GM2 gangliosidosis. A Beta-Hexosaminidase Beta Subunit Antibody can support studies of the HEXB protein and its contribution to lysosomal glycoconjugate degradation.
For additional information on HEXB biology and monoclonal antibody-based tissue validation, visit our Hexosaminidase B Antibody HEXB/7762 / Lysosomal Beta-Hexosaminidase Antibody page.
The stated application concentrations are suggested starting amounts. Titration of the HEXB Antibody / Beta-Hexosaminidase Beta Subunit Antibody may be required due to differences in protocols and secondary/substrate sensitivity.
Human partial recombinant protein (AA 381-556) was used as the immunogen for this HEXB antibody.
After reconstitution, the HEXB antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
HEXB antibody, beta-hexosaminidase beta subunit antibody, beta-hexosaminidase subunit beta antibody, Hex B antibody, beta-N-acetylhexosaminidase beta subunit antibody
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