Overview
| 别名 | Periaxin |
| 基因名 | PRX |
| UniProt ID | Q9BXM0 |
| 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 | 9N6-N7-J5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 154 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200-1:250 |
| 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 | Periaxin (PRX) is a scaffolding protein encoded by the PRX gene located on chromosome 19q13.13-q13.2 that plays a critical role in peripheral nerve function. The gene produces two alternatively spliced isoforms, L-periaxin and S-periaxin, both containing PDZ domains that facilitate protein-protein interactions essential for myelin maintenance. PRX functions as part of a dystroglycan complex in Schwann cells and is required for normal myelin sheath formation, maintenance, and remyelination after nerve injury. The protein regulates internodal length, enables saltatory nerve impulse transmission, and facilitates formation of Cajal bands and Schmidt-Lanterman incisures. Mutations in PRX cause autosomal recessive Dejerine-Sottas neuropathy (DSN) and Charcot-Marie-Tooth disease type 4F (CMT4F), both demyelinating peripheral neuropathies characterized by progressive weakness and sensory loss. Loss-of-function mutations delete critical protein domains, impairing cytoskeletal interactions and extracellular signal transmission necessary for Schwann cell function and myelin stability. |
检测原理