Application
Western blot analysis of human recombinant HEXB A chain fusion protein.| Product Name | HEXB A chain Antibody (H-40) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 408-447 mapping within an internal region of HEXB of human origin |
| Modification Notes | Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a Hexosaminidase B (HEXB), also designated b-hexosaminidase B, is a tetramer of two b-A and two b-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 b 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. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of HEXB A chain of human origin by WB, IP, IF and ELISA: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 408-447 mapping within an internal region of HEXB of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 408-447 mapping within an internal region of HEXB of human origin |
Application
Western blot analysis of human recombinant HEXB A chain fusion protein.| Application Notes | recommended for detection of HEXB A chain of human origin by WB, IP, IF and ELISA: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
