Overview
| 别名 | Actin; gamma-enteric smooth muscle; Alpha-actin-3; Gamma-2-actin; Smooth muscle gamma-actin) [Cleaved into: Actin; gamma-enteric smooth muscle; intermediate form] |
| 基因名 | ACTG2 |
| UniProt ID | P63267 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 8W9-S3-G7 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 41 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-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 | Actin gamma 2, smooth muscle (ACTG2) is a smooth muscle-specific isoform of actin that is essential for cell motility, cytoskeleton maintenance, and contractility in enteric tissues. It forms filaments that are critical components of the contractile apparatus in the intestine, bladder, and seminal vesicles. The protein interacts with regulatory partners such as emerin and leiomodin 1 to maintain smooth muscle function. Mutations in the ACTG2 gene, particularly heterozygous missense mutations, are the primary cause of visceral myopathy, a severe disorder characterized by life-threatening weakness of the bowel and bladder. These mutations disrupt actin function through various mechanisms, including the inhibition of polymerization, filament destabilization, and impaired actin-binding, leading to significant enteric smooth muscle dysfunction. |
检测原理