徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
TOR1AIP1 Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611661 |
| 100ul | WGD-Z-2611661 |
详细参数
| 别名 | Torsin-1A-interacting protein 1; Lamin-associated protein 1B; LAP1B |
| 基因名 | TOR1AIP1 |
| UniProt ID | Q5JTV8 |
| 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 | 3T2-Q2-O8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 66 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:4000; 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 | Torsin 1A interacting protein 1 (TOR1AIP1), also known as LAP1, is a type II integral membrane protein localized to the inner nuclear membrane. It plays a critical role in maintaining nuclear envelope integrity and chromatin organization by binding to A- and B-type lamins and activating the AAA+ ATPases torsinA and torsinB. Beyond its structural role, TOR1AIP1 participates in mitosis, centrosome positioning, and microtubule organization. Clinically, biallelic variants in TOR1AIP1 cause a spectrum of autosomal recessive nuclear envelopathies, including limb-girdle muscular dystrophy type 2Y, rigid-spine myopathy, and cardiomyopathy. Some cases also present with progeroid features and multisystem disease, characterized by early-onset muscle weakness and joint contractures. Additionally, dysregulated TOR1AIP1 expression has been implicated in the progression and prognosis of certain cancers, such as kidney renal clear cell carcinoma. |