产品说明
一般描述
This translocase is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome; an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms
免疫原
TIMM8A (NP_004076.1, 1 a.a. ~ 97 a.a) full-length human protein.
Sequence
MDSSSSSSAAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
外形
Solution in phosphate buffered saline, pH 7.4
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
| NACRES | NA.41 |
产品性质
| 质量水平 | 100 |
| 生物来源 | mouse |
| 偶联物 | unconjugated |
| 抗体形式 | purified immunoglobulin |
| antibody product type | primary antibodies |
| 克隆 | polyclonal |
| 形式 | buffered aqueous solution |
| 分子量 | antigen ~11 kDa |
| species reactivity | human |
| technique(s) | indirect immunofluorescence: suitable western blot: 1 μg/mL |
| NCBI登记号 | NM_004085 |
| UniProt登记号 | O60220 |
| 运输 | dry ice |
| 储存温度 | −20℃ |
| Gene Information | human ... TIMM8A(1678) |
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 |
