| 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 | Junctophilin-3, JP-3, Junctophilin type 3, Jph3, Jp3 |
| Entrez GeneID | 57340 |
| WB Predicted band size | 81.2kDa |
| 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 Junctophilin 3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 549-578 amino acids from the Central region of human Junctophilin 3. |
| Formulation | Purified antibody in TBS with 0.05% sodium azide. |
+ +
以下是3篇与Junctophilin 3(JPH3)抗体相关的文献概览：
---
1. **"Junctophilin-3 is a novel biomarker for Huntington's disease-like disorders"**
- **作者**: Holmes SE et al.
- **摘要**: 该研究利用JPH3抗体检测亨廷顿病样患者脑组织中的蛋白表达异常，发现JPH3与神经元钙信号失调相关，提示其作为神经退行性疾病的潜在生物标记物。
---
2. **"Immunolocalization of junctophilin-3 in cardiac hypertrophy models"**
- **作者**: Chen B et al.
- **摘要**: 通过免疫组化和Western blot分析，发现JPH3抗体在小鼠心肌肥厚模型中显示蛋白表达下调，表明JPH3在维持心肌细胞T管结构及钙稳态中的关键作用。
---
3. **"Junctophilin-3 deficiency disrupts synaptic transmission in cerebellar Purkinje cells"**
- **作者**: Ito-Ishida A et al.
- **摘要**: 研究使用JPH3抗体在小脑浦肯野细胞中定位蛋白，发现JPH3缺失导致突触连接异常和运动协调障碍，揭示其在神经元兴奋-收缩耦联中的功能。
---
注：以上文献为示例性质，实际引用时需核对真实来源及出版信息。
Junctophilin 3 (JPH3) is a member of the junctophilin family, which plays a critical role in maintaining the structural and functional coupling between the plasma membrane and endoplasmic/sarcoplasmic reticulum in excitable cells. Specifically, JPH3 is highly expressed in the brain and contributes to the formation of junctional membrane complexes, essential for precise calcium signaling and excitation-contraction coupling. Its N-terminal domain binds the plasma membrane, while the C-terminal region anchors to the endoplasmic reticulum, stabilizing communication between these compartments.
Antibodies targeting JPH3 are vital tools for studying its expression, localization, and interaction partners in both physiological and pathological contexts. Research has linked JPH3 dysfunction to neurodegenerative disorders, particularly Huntington’s disease-like 2 (HDL2), a condition caused by CTG/CAG repeat expansions in the *JPH3* gene. Antibodies against JPH3 enable detection of protein mislocalization, aggregation, or reduced expression in disease models, aiding mechanistic insights into neuronal calcium dysregulation and associated degeneration.
These antibodies are typically generated using immunogenic peptides or recombinant protein fragments, validated for applications such as Western blotting, immunohistochemistry, and immunofluorescence. Their specificity helps distinguish JPH3 from other junctophilin isoforms (e.g., JPH1. JPH2) and assess tissue-specific expression patterns. Ongoing studies leverage JPH3 antibodies to explore therapeutic strategies targeting membrane contact sites in neurological and muscular disorders, underscoring their importance in translational research.
×
