徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
RNASEH2C Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611844 |
| 100ul | WGD-Z-2611844 |
详细参数
| 别名 | Ribonuclease H2 subunit C; RNase H2 subunit C; Aicardi-Goutieres syndrome 3 protein; AGS3; RNase H1 small subunit; Ribonuclease HI subunit C |
| 基因名 | RNASEH2C |
| UniProt ID | Q8TDP1 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 4Y9-J1-L4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 17 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 | Ribonuclease H2 subunit C (RNASEH2C) is the non-catalytic component of the heterotrimeric RNase H2 complex, which degrades RNA:DNA hybrids and removes misincorporated ribonucleotides to maintain genomic stability. Structurally, RNASEH2C is integral to the formation of the complex's triple beta-barrel core, ensuring the stability and enzymatic activity of the catalytic subunit. Biallelic loss-of-function mutations in RNASEH2C cause Aicardi-Goutières syndrome type 3 (AGS3), an autoinflammatory encephalopathy characterized by severe neurological dysfunction, intracranial calcifications, and leukodystrophy. These defects lead to the accumulation of endogenous nucleic acids, triggering aberrant type I interferon-mediated immune responses. |