Overview
| 别名 | Exosome complex component 10; Autoantigen PM/Scl 2; P100 polymyositis-scleroderma overlap syndrome-associated autoantigen; Polymyositis/scleroderma autoantigen 100 kDa; PM/Scl-100; Polymyositis/scleroderma autoantigen 2 |
| 基因名 | EXOSC10 |
| UniProt ID | Q01780 |
| 反应种属 | Human |
| 应用 | WB,IP,CHIP |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 9I7-B3-R8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 100 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IP-1:100; CHIP-1:100 |
| 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 | Exosome component 10 (EXOSC10), also known as PM/Scl-100, is a catalytic subunit of the RNA exosome complex, possessing 3'-5' exoribonuclease activity. It is essential for RNA processing, degradation, and quality control in both the nucleus and cytoplasm. In the nucleus, EXOSC10 is involved in the maturation of ribosomal RNA and small nuclear RNAs, while in the cytoplasm, it regulates the turnover of unstable mRNAs, particularly those containing AU-rich elements. Beyond RNA metabolism, it contributes to DNA repair via homologous recombination and is vital for germ cell development. Clinically, EXOSC10 is a prominent autoantigen in systemic autoimmune diseases, most notably scleromyositis, which encompasses features of both diffuse scleroderma and polymyositis. Mutations in the EXOSC10 gene are also linked to severe developmental and neurological disorders. |
检测原理