| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | TSD |
| WB Predicted band size | 61 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Fusion protein of human HEXA |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于HEXA抗体的3篇代表性文献的简要概括(文献标题与内容为示例，具体文献需通过学术数据库查询)：
1. **文献名称**: "Development of a monoclonal antibody specific for human HEXA protein for diagnostic applications"
**作者**: Smith J, et al.
**摘要**: 本研究报道了一种针对人类β-己糖胺酶A(HEXA)的单克隆抗体制备方法，该抗体能够特异性识别HEXA的α亚基。实验验证了其在泰-萨克斯病(Tay-Sachs disease)患者细胞中HEXA蛋白缺失的检测能力，为遗传病诊断提供了新工具。
2. **文献名称**: "Immunohistochemical analysis of HEXA expression in mouse models of lysosomal storage disorders"
**作者**: Lee C, et al.
**摘要**: 通过HEXA抗体的免疫组化分析，本研究揭示了HEXA蛋白在小鼠脑组织中的分布模式，并发现其在神经节苷脂代谢异常模型中的表达下调，为研究溶酶体疾病的病理机制提供了依据。
3. **文献名称**: "A novel ELISA using anti-HEXA antibody for rapid screening of Tay-Sachs carriers"
**作者**: Gonzalez R, et al.
**摘要**: 研究团队基于抗HEXA抗体开发了一种高灵敏度ELISA检测方法，可定量分析血液中HEXA酶活性，用于泰-萨克斯病携带者的快速筛查，临床样本验证显示其特异性和灵敏度均超过传统生化检测法。
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**备注**：以上文献信息为示例，实际文献需通过PubMed、Web of Science等平台检索关键词(如"HEXA antibody"、"hexosaminidase A immunoassay")。近年研究可能涉及HEXA抗体在基因治疗监测或新生儿筛查中的应用拓展。
HEXA antibodies are immunological tools specifically designed to target the β-subunit of hexosaminidase A (HEXA), a critical lysosomal enzyme encoded by the *HEXA* gene. HEXA, in combination with the β-subunit (HEXB), forms the heterodimeric enzyme β-hexosaminidase A, which is essential for degrading GM2 gangliosides in neuronal cells. Mutations in *HEXA* disrupt enzyme function, leading to GM2 accumulation and Tay-Sachs disease—a fatal neurodegenerative disorder.
HEXA antibodies are primarily used in research and diagnostics to study enzyme expression, localization, and activity. They enable detection of HEXA protein levels via techniques like Western blotting, immunohistochemistry, and ELISA, aiding in the identification of genetic deficiencies linked to Tay-Sachs. These antibodies also facilitate carrier screening, prenatal testing, and mechanistic studies of disease pathology.
Developed in various host species (e.g., mouse, rabbit), HEXA antibodies are typically monoclonal or polyclonal, with specificity validated against recombinant or native proteins. Their application extends to evaluating therapeutic interventions, such as gene therapy or enzyme replacement, in preclinical models. By elucidating HEXA's role in lysosomal function and disease, these antibodies contribute to advancing understanding of GM2 gangliosidoses and developing targeted treatments.
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