Overview
| 别名 | Superkiller complex protein 3; Ski3; Tetratricopeptide repeat protein 37; TPR repeat protein 37; Tricho-hepatic-enteric syndrome protein; Thespin |
| 基因名 | SKIC3 |
| UniProt ID | Q6PGP7 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 9M1-I8-V9 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 175 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000 |
| 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 | SKI3 subunit of superkiller complex (SKIC3) is a cofactor essential for the RNA exosome-mediated 3'–5' mRNA decay. Containing multiple tetratricopeptide repeat (TPR) motifs, SKIC3 serves as a scaffold to position the helicase within the complex, channeling aberrant or ribosome-bound mRNAs to the exosome for degradation. This process is critical for mRNA surveillance and ribosome-associated quality control. Ubiquitously expressed, SKIC3 is vital for cellular homeostasis. Biallelic loss-of-function variants in SKIC3 are the cause of trichohepatoenteric syndrome (THES) type 1, a multisystem disorder characterized by intractable diarrhea, immune dysfunction, and hair abnormalities. |
检测原理