Trial Size Available
Carrier-Free Available
SDS-PAGE Analysis of Purified HEXB Mouse Monoclonal Antibody (HEXB/7762). Confirmation of Purity and Integrity of Antibody.
Formalin-fixed, paraffin-embedded human placenta stained with HEXB Mouse Monoclonal Antibody (HEXB/7762). Inset: PBS instead of primary antibody; secondary only negative control.
| Document Name | |
|---|---|
| Datasheet | Download Here |
| Material Safety Data Sheet | Download Here |
| Applications | Tested Dilutions | Protocol | Note |
|---|---|---|---|
| Immunohistochemistry (IHC) | 1-2ug/ml | IHC Protocol | 30 min at RT. Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes |
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Hexosaminidase B (HEXB), also designated β-hexosaminidase B, is a hexosaminidase B (HEXB), also designated β-hexosaminidase B, is a tetramer of two β-A and two β-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the β 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.
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