| 產(chǎn)品編號(hào) | bsm-51197M-BF594 |
| 英文名稱 | Mouse Anti-PLP1/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的小鼠抗髓磷酯髓鞘蛋白1單克隆抗體 |
| 別 名 | Proteolipid Protein 1; Lipophilin; PLP; Spastic Paraplegia 2, Uncomplicated; Major Myelin Proteolipid Protein; Pelizaeus-Merzbacher Disease; Myelin Proteolipid Protein; PLP/DM20; GPM6C; HLD1; MMPL; SPG2; PMD; MYPR_RAT; |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 神經(jīng)生物學(xué) 細(xì)胞粘附分子 |
| 抗體來(lái)源 | Mouse |
| 克隆類型 | Monoclonal |
| 克 隆 號(hào) | 4E7 |
| 交叉反應(yīng) | (predicted: Human, Rat, ) |
| 產(chǎn)品應(yīng)用 |
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 30kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from rat PLP1 |
| 亞 型 | IgM |
| 純化方法 | affinity purified by Protein AGL |
| 儲(chǔ) 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: PLP is a major constituent of myelin. The two isoforms of the myelin proteolipid protein, PLP and DM20, are very hydrophobic integral membrane proteins that account for about half of the protein content of adult CNS myelin. A mutation in the gene which encodes PLP is linked to Pelizaeus-Merzbacher disease (PMD), a chronic infantile type of diffuse cerebral sclerosis. The gene which encodes PLP maps to human chromosome Xq22. The glycoprotein zero (also designated P-zero or myelin peripheral protein) is the primary structural protein of peripheral myelin, and accounts for more than 50% of the protein present in the peripheral nerve sheath. Zero is an integral membrane glycoprotein. Expression of zero is restricted to Schwann cells. The gene which encodes zero maps to human chromosome 1q22. PMP22 (peripheral myelin protein 22) is a growth-regulated membrane protein which is expressed by Schwann cells and is localized primarily in compact peripheral nervous system myelin. The gene which encodes PMP22 maps to human chromosome 17p11.2. Function: This is the major myelin protein from the central nervous system. It plays an important role in the formation or maintenance of the multilamellar structure of myelin. Subunit: Belongs to the myelin proteolipid protein family. Subcellular Location: Membrane; Multi-pass membrane protein. DISEASE: Defects in PLP1 are the cause of leukodystrophy hypomyelinating type 1 (HLD1) [MIM:312080]; also known as Pelizaeus-Merzbacher disease. HLD1 is an X-linked recessive dysmyelinating disorder of the central nervous system in which myelin is not formed properly. It is characterized clinically by nystagmus, spastic quadriplegia, ataxia, and developmental delay. Defects in PLP1 are the cause of spastic paraplegia X-linked type 2 (SPG2) [MIM:312920]. SPG2 is characterized by spastic gait and hyperreflexia. In some patients, complicating features include nystagmus, dysarthria, sensory disturbance, mental retardation, optic atrophy. Similarity: Belongs to the myelin proteolipid protein family. Database links: Entrez Gene: 5354 Human Entrez Gene: 18823 Mouse Omim: 300401 Human SwissProt: P60201 Human SwissProt: P60202 Mouse Unigene: 49691 Cow Unigene: 1787 Human Unigene: 1268 Mouse Unigene: 4550 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
