HEXB Antibody / Hexosaminidase B
| Code | Size | Price |
|---|
| NSJ-V5089SAF-100UG | 100 ug | £534.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Mouse
Antibody Isotype: Mouse IgG2, kappa
Antibody Clonality: Monoclonal
Antibody Clone: HEXB/7762
Regulatory Status: RUO
Target Species: Human
Application: Immunohistochemistry- Paraffin Embedded (IHC-P)
Storage:
Aliquot the Hexosaminidase B antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.
Documents
Further Information
Application Details:
Immunohistochemistry (FFPE): 1-2ug/ml for 30 min at RT
Description:
Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a tetramer of two beta-A and two beta-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 beta 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.
Format:
Purified
Formulation:
1 mg/ml in 1X PBS; BSA free, sodium azide free
Immunogen:
A recombinant fragment of the human protein was used as the immunogen for the Hexosaminidase B antibody.
Limitation:
This Hexosaminidase B antibody is available for research use only.
Localization:
Lysosome
Purity:
Protein A/G affinity
Uniprot #:
P07686