Thermo Fisher Scientific HEXB Monoclonal Antibody (HEXB/7762)
HEXB 단백질을 인식하는 마우스 단클론 항체로, 인체 조직의 면역조직화학(IHC) 분석에 적합합니다. 리소좀 내 HEXB 단백질 검출에 사용되며, 샌드호프병 연구 등 HEXB 관련 질환 연구에 활용 가능합니다. 단백질 A/G로 정제된 액상 형태입니다.
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Applications and Tested Dilution
- Immunohistochemistry (Paraffin) (IHC (P)): 1–2 µg/mL
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Mouse / IgG2, kappa |
| Class | Monoclonal |
| Type | Antibody |
| Clone | HEXB/7762 |
| Immunogen | Recombinant fragment human HEXB protein (exact sequence is proprietary) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 200 µg/mL |
| Purification | Protein A/G |
| Storage Buffer | PBS with 0.05% BSA |
| Contains | 0.05% sodium azide |
| Storage Conditions | 4°C |
| Shipping Conditions | Ambient (domestic); Wet ice (international) |
Product Specific Information
- Positive Control: Human kidney or lung
- Cellular Location: Lysosome
Target Information
Hexosaminidase B (HEXB), also known as β-hexosaminidase B, is a tetramer composed of two β-A and two β-B chains, localized in the lysosomes of cells.
Mutations in the HEXB gene cause Sandhoff disease (GM2-gangliosidosis type II) by disrupting the activity of HEXB and HEXA enzymes, leading to accumulation of GM2 ganglioside in the brain and progressive neurodegeneration.
Sandhoff disease is an autosomal recessive disorder and is clinically similar to Tay-Sachs disease, though generally more severe.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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