Overview
| 别名 | PDZ and LIM domain protein 3; Actinin-associated LIM protein; Alpha-actinin-2-associated LIM protein |
| 基因名 | PDLIM3 |
| UniProt ID | Q53GG5 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 7G7-O6-O9 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 39 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 | PDZ and LIM domain 3 (PDLIM3) is a cytoskeletal scaffold protein that is highly expressed in cardiac and skeletal muscle. It localizes to Z-discs and intercalated discs, where it binds α-actinin-2 via its N-terminal PDZ domain to organize actin filament arrays and maintain the structural integrity of the contractile apparatus. PDLIM3 is essential for the proper assembly and function of striated muscle. Clinically, mutations in PDLIM3 are associated with inherited cardiac arrhythmia syndromes, including Brugada syndrome 6 and atrial standstill 1. Furthermore, aberrant alternative splicing of this protein has been linked to myotonic dystrophy, suggesting that disruption of its scaffolding function contributes to the development of cardiomyopathy and skeletal muscle pathology. |
检测原理