Overview
| 别名 | Gephyrin [Includes: Molybdopterin adenylyltransferase; MPT adenylyltransferase; Domain G); Molybdopterin molybdenumtransferase; MPT Mo-transferase; Domain E] |
| 基因名 | Gephyrin |
| UniProt ID | Q9NQX3 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1G6-J9-L9 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 79 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Gephyrin (Gephyrin) is a cytoplasmic, microtubule-associated scaffolding protein essential for organizing the inhibitory postsynaptic density. It functions by anchoring and clustering inhibitory glycine and GABA_A receptors at postsynaptic sites, linking them to the underlying cytoskeleton. Structurally, gephyrin is a modular protein comprising an N-terminal G domain, a C-terminal E domain that binds receptor subunits, and an intrinsically disordered linker that facilitates dynamic oligomerization and synaptic plasticity. Beyond its role in neurotransmission, gephyrin is required for molybdenum cofactor biosynthesis, thereby connecting synaptic function to cellular metabolism. Clinically, pathogenic variants in the GPHN gene are associated with epileptic encephalopathy, hyperekplexia, and intellectual disability due to impaired receptor clustering. Additionally, autoantibodies against gephyrin are identified in stiff-person spectrum disorders, highlighting its involvement in autoimmune neurological conditions. |
检测原理