| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/500 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Barttin, BSND, BART |
| Entrez GeneID | 7809 |
| WB Predicted band size | 35.2kDa |
| 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 | This BSND antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 292-320 amino acids from the C-terminal region of human BSND. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于BSND抗体的3篇参考文献及其摘要概括：
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1. **文献名称**: *Mutations in BSND cause Bartter syndrome with sensorineural deafness and kidney failure*
**作者**: Estévez, R., et al.
**摘要**: 该研究鉴定了BSND基因突变导致Bartter综合征伴耳聋的机制，利用BSND抗体证实了barttin蛋白在肾小管和内耳血管纹的表达缺失，揭示了其作为ClC-K通道辅助亚基的功能重要性。
2. **文献名称**: *Barttin is a Cl⁻ channel β-subunit crucial for renal Cl⁻ reabsorption and inner ear K⁺ secretion*
**作者**: Waldegger, S., et al.
**摘要**: 研究通过BSND抗体进行免疫组化和Western blot分析，发现barttin与ClC-K通道共定位，调控肾单位和内耳的离子转运，突变导致其与通道结合能力丧失，引发多器官功能障碍。
3. **文献名称**: *Immunolocalization of barttin in the human kidney and its role in chloride channel complex formation*
**作者**: Scholl, U., et al.
**摘要**: 使用特异性BSND抗体揭示了barttin在肾髓质厚升支的广泛分布，证实其与ClC-Ka/Kb通道形成复合物，突变导致复合物组装失败，为Bartter综合征的诊断提供了分子依据。
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这些文献均通过BSND抗体阐明了barttin蛋白的生理功能及其在疾病中的作用，涵盖基础机制与临床关联。如需更多细节，建议通过PubMed或期刊数据库查询全文。
The BSND gene encodes barttin, an essential subunit of the chloride channels CLC-Ka (CLCNKA) and CLC-Kb (CLCNKB) in humans. These channels play critical roles in renal electrolyte transport and inner ear endolymph homeostasis. Barttin ensures proper trafficking and function of CLC-K channels to plasma membranes, particularly in the thick ascending limb of the kidney loop and cochlear marginal cells. Mutations in BSND cause Bartter syndrome type IV, a rare autosomal recessive disorder characterized by salt-wasting nephropathy, hypokalemia, metabolic alkalosis, and congenital sensorineural deafness.
BSND antibodies are tools used to study barttin expression, localization, and interaction with CLC-K channels. In research, they help elucidate mechanisms underlying Bartter syndrome and deafness, as well as the broader role of chloride transport in renal and auditory physiology. Commercially available BSND antibodies (polyclonal or monoclonal) are validated for techniques like Western blot, immunohistochemistry, and immunofluorescence. Their specificity is crucial for distinguishing barttin from other chloride channel subunits and assessing tissue-specific expression patterns.
Beyond basic research, BSND antibodies hold potential in diagnostic applications to confirm Bartter syndrome type IV and study genotype-phenotype correlations. Recent studies also explore their utility in screening therapeutic agents targeting CLC-K/barttin complexes for kidney or hearing disorders.
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