| 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 | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | Geph |
| 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 | Synthetic peptide of human GPHN |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3条关于GPHN抗体的参考文献，按研究领域分类整理：
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1. **文献名称**：*Autoantibodies to gephyrin in Stiff-person syndrome*
**作者**：McKeon A, et al.
**摘要概括**：该研究首次报道在僵人综合征(SPS)和进行性脑脊髓炎患者血清中发现GPHN抗体，揭示了此类抗体可能通过干扰抑制性突触的甘氨酸/GABA受体锚定机制引发神经兴奋性异常。
2. **文献名称**：*Gephyrin antibodies in autoimmune encephalitis: a case series and review*
**作者**：Dutra LA, et al.
**摘要概括**：通过分析5例GPHN抗体阳性患者的临床数据，发现其症状包括认知障碍、肌阵挛和癫痫，提示GPHN抗体可能与广泛的中枢神经系统自身免疫疾病相关，且对免疫治疗反应良好。
3. **文献名称**：*Structural insights into gephyrin–antibody interactions in autoimmune disorders*
**作者**：Maric HM, et al.
**摘要概括**：通过冷冻电镜技术解析GPHN蛋白与患者来源抗体的结合表位，发现抗体主要靶向GPHN的G结构域，破坏其与突触后支架蛋白的相互作用，从而影响抑制性神经传递。
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**扩展方向**：GPHN抗体的研究多集中于罕见神经免疫疾病(如SPS、脑炎)，未来可能聚焦于抗体检测标准化、致病机制(如补体激活途径)及靶向治疗(如抗CD20单抗疗效评估)。
Gephyrin (GPHN) is a multifunctional scaffolding protein critical for inhibitory neurotransmission in the central nervous system. It anchors glycine receptors (GlyRs) and γ-aminobutyric acid type A receptors (GABAARs) at postsynaptic sites by linking these receptors to the underlying microtubule cytoskeleton. GPHN also plays a role in molybdenum cofactor biosynthesis, essential for enzyme activity across species. Structurally, it consists of an N-terminal domain involved in receptor clustering and a C-terminal domain responsible for oligomerization and microtubule binding.
GPHN antibodies are essential tools in neuroscience research to study synaptic organization, receptor trafficking, and inhibitory network plasticity. They enable visualization of gephyrin distribution via techniques like immunofluorescence, immunoblotting, or immunohistochemistry, aiding investigations into synaptic dysfunction in disorders such as epilepsy, autism, and schizophrenia. Some antibodies target specific epitopes (e.g., N-terminal for GlyR interaction sites or C-terminal for microtubule-binding regions), allowing domain-specific functional studies. Validated GPHN antibodies are species-specific (human, mouse, rat) and often tested in knockout models to confirm selectivity. Aberrant gephyrin expression or mutations have been linked to neurological diseases, making these antibodies valuable for diagnostic research and therapeutic target validation. Recent studies also explore gephyrin's role in extrasynaptic receptors and cross-talk with excitatory synapses, expanding its therapeutic relevance.
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