产品说明
一般描述
Translocase of inner mitochondrial membrane 8A (TIMM8A) 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. TIMM8A gene is located on human chromosome Xq22. It is a 11 kDa protein. It belongs to a family of evolutionary conserved proteins, which are arranged in hetero-oligomeric complexes in the mitochondrial intermembrane space.
免疫原
TIMM8A (NP_004076, 9 a.a. ~ 97 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
AAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
应用
Monoclonal Anti-TIMM8A antibody produced in mouse has been used in immunoblotting.
生化/生理作用
Translocase of inner mitochondrial membrane 8A (TIMM8A) is involved in the dynamin-1-like protein (Drp1)-mediated mitochondrial fission during programmed cell death. It interacts with TIMM13 and aids the import of TIMM23 into mitochondria.{152)
外形
Solution in phosphate buffered saline, pH 7.4
法律信息
GenBank is a registered trademark of United States Department of Health and Human Services
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 | 2F11, monoclonal |
| 形式 | buffered aqueous solution |
| species reactivity | human |
| technique(s) | immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable indirect ELISA: suitable western blot: 1-5 μg/mL |
| 同位素/亚型 | IgG2aκ |
| GenBank® accession no. | 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 | 12 - Non Combustible Liquids |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
| 个人防护装备 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |
