| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/5000 | Human,Mouse,Rat |
| Aliases | ED2, EDH, HED, CX30, HED2, DFNA3, ECTD2, DFNA3B, DFNB1B |
| 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 |
| Immunogen | Synthetic peptide of human GJB6 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于GJB6抗体的3篇示例性参考文献(内容基于领域知识模拟，实际文献请通过学术数据库查询)：
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1. **文献名称**: "GJB6基因突变导致先天性耳聋的分子机制研究"
**作者**: Smith A, et al.
**摘要**: 本研究通过Western blot和免疫组化技术，利用GJB6特异性抗体分析耳蜗组织中的蛋白表达，发现GJB6突变导致间隙连接功能异常，影响耳蜗钾离子循环，从而引发听力障碍。
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2. **文献名称**: "Development and Validation of a Novel Anti-GJB6 Antibody for Skin Disease Diagnostics"
**作者**: Zhang L, et al.
**摘要**: 研究团队开发了一种高特异性兔源GJB6多克隆抗体，经ELISA和免疫荧光验证，成功应用于Clouston综合征患者皮肤活检样本的蛋白定位分析，为遗传性皮肤病诊断提供工具。
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3. **文献名称**: "Connexin 30 (GJB6) and Connexin 26 (GJB2) Cooperativity in Epidermal Homeostasis"
**作者**: Garcia M, et al.
**摘要**: 通过双标记免疫荧光技术结合GJB6和GJB2抗体，揭示两种连接蛋白在表皮细胞中的共定位及功能协同性，阐明其在维持皮肤屏障中的作用机制。
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**备注**：以上文献信息为模拟示例，实际研究需参考PubMed、Web of Science等平台的具体论文。如需真实文献，建议检索关键词如"GJB6 antibody application"或"Connexin 30 antibody"。
GJB6 antibody targets the gap junction protein beta-6 (GJB6), also known as connexin 30 (Cx30), which is encoded by the *GJB6* gene. Gap junction proteins form intercellular channels facilitating direct communication between adjacent cells, enabling the exchange of ions, metabolites, and signaling molecules. These channels are critical for maintaining tissue homeostasis, particularly in organs like the skin, inner ear, and brain.
Mutations in *GJB6* are linked to several disorders. Notably, autosomal-dominant mutations cause hidrotic ectodermal dysplasia (Clouston syndrome), characterized by hair loss, nail dystrophy, and hyperkeratosis. Recessive mutations in *GJB6*, often in combination with *GJB2* (connexin 26), are associated with nonsyndromic hearing loss. GJB6 antibodies are thus valuable tools for studying these conditions, aiding in protein localization, expression analysis, and understanding pathogenic mechanisms.
In research, GJB6 antibodies are used in techniques like immunohistochemistry, Western blotting, and immunofluorescence to assess protein distribution in tissues such as the epidermis, cochlea, and placenta. They also help investigate the role of Cx30 in skin barrier function, cochlear potassium recycling, and neural activity. Clinically, these antibodies may assist in diagnosing genetic disorders or validating gene therapy outcomes. However, specificity and cross-reactivity with other connexins require careful validation. Overall, GJB6 antibodies contribute to both basic research and translational studies of connexin-related diseases.
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