HEXB (E9X5S) Rabbit mAb #33663
- WB
- IHC
- IF
Supporting Data
| REACTIVITY | M R |
| SENSITIVITY | Endogenous |
| MW (kDa) | 52, 70 |
| Source/Isotype | Rabbit IgG |
Application Key:
- WB-Western Blotting
- IHC-Immunohistochemistry
- IF-Immunofluorescence
Species Cross-Reactivity Key:
- M-Mouse
- R-Rat
Product Information
Product Usage Information
| Application | Dilution |
|---|---|
| Western Blotting | 1:1000 |
| Immunohistochemistry (Paraffin) | 1:100 - 1:400 |
| Immunofluorescence (Frozen) | 1:800 - 1:3200 |
| Immunofluorescence (Immunocytochemistry) | 1:200 |
Storage
For a carrier free (BSA and azide free) version of this product see product #28112.
Protocol
Specificity / Sensitivity
Species Reactivity:
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Pro300 of mouse HEXB protein.
Background
β-hexosaminidase (Hex) is an important lysosomal enzyme system that degrades various cellular substrates, such as oligosaccharides, glycosaminoglycans, and glycolipids. It is encoded by two genes, HEXA and HEXB, respectively, and these subunits dimerize to form β-hexosaminidase A (HexA; αβ) and β-hexosaminidase B (HexB; ββ) (1). Loss-of-function mutations result in ganglioside (GM2) accumulation and progressive neurodegenerative diseases, such as Sandhoff disease (SD) and Tay-Sachs disease (TSD) (1). HexB knockout mice display, similarly to human patients, a near-normal phenotype at birth but quickly develop muscle weakness, rigidity, and motor deterioration, typically leading to death at approximately four months of age (2). It has been shown that loss of HEXB leads to reduction of early neuronal migration and differentiation in the embryonic cerebral cortices of these mice (3). Hex also plays an important role in cancer, being a new potential biomarker in laryngeal cancer. Furthermore, higher expression of HEXA and HEXB is associated with poor prognosis in glioblastoma multiforme (GBM) patients (1).
限制使用
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