Overview
| 别名 | Alpha-actinin-2; Alpha-actinin skeletal muscle isoform 2; F-actin cross-linking protein |
| 基因名 | Sarcomeric alpha Actinin |
| UniProt ID | P35609 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 5T9-P9-S8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 103 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200 |
| 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 | Actinin alpha 2 (Sarcomeric alpha Actinin) is a major actin-binding cytoskeletal protein belonging to the spectrin superfamily, primarily expressed in the Z-discs of skeletal and cardiac muscle. It forms antiparallel homodimers that crosslink actin filaments and titin, providing a structural scaffold essential for sarcomeric integrity, force transmission, and myofibril anchoring. Beyond its structural role, it scaffolds various signaling complexes, including ion channels like Kv1.5 and enzymes such as calcineurin, thereby supporting excitation-contraction coupling and synaptic stability. Its activity is dynamically modulated by phospholipids and calcium levels. Clinically, mutations in the ACTN2 gene are well-established causes of Dilated Cardiomyopathy 1AA, characterized by progressive heart failure, and Congenital Myopathy 8, which presents as early-onset muscle weakness and hypotonia. |
检测原理