| Product Name | Anti-HEXB Antibody [HEXB/7762] - BSA and Azide free |
|---|---|
| Description | Mouse monoclonal [HEXB/7762] antibody to HEXB. |
| Host | Mouse |
| Clonality | Monoclonal |
| Clone | HEXB/7762 |
| Conjugate | Unconjugated |
| Immunogen | Recombinant fragment human HEXB protein. The exact sequence is proprietary. |
| Isotype | IgG2 |
| Molecular Weight | 63 kDa |
| Target | HEXB |
| Specificity | Hexosaminidase B (HEXB), also designated subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe. |
| Reactivity | Human |
| Applications | IHC-P |
| Working Dilutions | IHC: 1-2 µg/ml |
| Form | Liquid |
| Diluent Buffer | Supplied in 10mM Phosphate Buffered Saline; without Sodium Azide and carrier free. |
| Concentration | 1 mg/ml |
| Storage | Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. |
| Supplier | Antibodies.com |
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