徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
TSC1 Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611869 |
| 100ul | WGD-Z-2611869 |
详细参数
| 别名 | Hamartin; Tuberous sclerosis 1 protein |
| 基因名 | Hamartin |
| UniProt ID | Q92574 |
| 反应种属 | Human |
| 应用 | WB,IP,CHIP |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1V6-L4-M5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 129 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 | TSC complex subunit 1 (TSC1), also known as hamartin, is a tumor suppressor protein that plays a crucial role in regulating cell growth and proliferation. TSC1 forms a multi-protein complex with TSC2 (tuberin) and TBC1D7, creating an elongated, scorpion-like structure where TSC1’s coiled-coil backbone runs along the TSC2 dimer, breaking its symmetry and facilitating complex assembly. This complex acts as a GTPase-activating protein (GAP) for the small GTPase Rheb, thereby negatively regulating the mammalian target of rapamycin complex 1 (mTORC1), a central controller of anabolic cell growth. TSC1 also functions as a co-chaperone for Hsp90, inhibiting its ATPase activity and stabilizing client proteins, including TSC2, by preventing their ubiquitination and proteasomal degradation. Mutations in TSC1 disrupt these regulatory functions, leading to diseases such as tuberous sclerosis complex—a genetic disorder characterized by benign tumor growth in multiple organs—and lymphangioleiomyomatosis. |