产品说明
一般描述
This gene encodes an integral membrane protein that is a major component of myelin in the peripheral nervous system. Various mutations of this gene are causes of Charcot-Marie-Tooth disease Type IA, Dejerine-Sottas syndrome, and hereditary neuropathy with liability to pressure palsies. Alternative splicing of this gene results in three transcript variants that encode the same protein. (provided by RefSeq)
免疫原
PMP22 (AAH19040, 25 a.a. ~ 114 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
VSQWIVGNGHATDLWQNCSTSSSGNVHHCFSSSPNEWLQSVQATMILSIIFSILSLFLFFCQLFTLTKGGRFYITGIFQILAGLCVMSAA
生化/生理作用
Peripheral myelin protein 22 (PMP22) plays a vital role in myelination during peripheral nerve development. It also has a role in cell-cell interactions, cell proliferation, maintenance of axons and the determination of myelin thickness and stability. Aberrations or mutations in the PMP22 gene lead to heritable demyelinating peripheral neuropathies, such as Charcot-Marie-tooth disease type IA (CMT1A) and Dejerine-Sottas syndrome. Overexpression of PMP22 might contribute to the development of chronic myeloid leukemia (CML).
外形
Solution in phosphate buffered saline, pH 7.4
基本信息
| NACRES | NA.41 |
产品性质
| 生物来源 | mouse |
| 偶联物 | unconjugated |
| 抗体形式 | purified immunoglobulin |
| antibody product type | primary antibodies |
| 克隆 | 3G10, monoclonal |
| 形式 | buffered aqueous solution |
| 分子量 | antigen ~35.9 kDa |
| species reactivity | human |
| technique(s) | indirect ELISA: suitable |
| 同位素/亚型 | IgG2bκ |
| NCBI登记号 | BC019040 |
| UniProt登记号 | Q01453 |
| 运输 | dry ice |
| 储存温度 | −20℃ |
| Gene Information | human ... PMP22(5376) |
安全信息
| 储存分类代码 | 13 - Non Combustible Solids |
| WGK | WGK 1 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
Sigma-Aldrich
m.cnreagent.com
