Overview
| 别名 | Myelin proteolipid protein; PLP; Lipophilin |
| 基因名 | PLP1 |
| UniProt ID | P60201 |
| 反应种属 | Human,Mouse,Rat |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 6V9-K5-I9 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 30 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:100-1:200 |
| 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 | Proteolipid protein 1 (PLP1) is the primary transmembrane protein of the central nervous system myelin, accounting for approximately half of its total protein content. It possesses a tetraspan topology that is essential for myelin compaction, stabilization, and the maintenance of the multilamellar structure. PLP1 facilitates the adherence of myelin layers through extracellular self-binding and interacts with lipid rafts rich in cholesterol and sphingomyelin. It also produces the DM20 isoform via alternative splicing, which is prevalent in peripheral nerves. Mutations, duplications, or deletions of the PLP1 gene lead to severe X-linked hypomyelinating leukodystrophies, most notably Pelizaeus-Merzbacher disease (PMD) and spastic paraplegia type 2 (SPG2), which are characterized by nystagmus, ataxia, and cognitive impairment due to disrupted oligodendrocyte development and axonal survival. |
检测原理