Overview
| 别名 | Telethonin; Titin cap protein |
| 基因名 | TCAP |
| UniProt ID | O15273 |
| 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 | 5N8-E5-N3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 19 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 | Titin-cap (TCAP), also known as telethonin, is a muscle-specific protein localized to the sarcomeric Z-disc in cardiac and skeletal myocytes. It functions as a critical structural adapter by binding the N-terminal Z1-Z2 domains of titin in a unique palindromic arrangement, thereby anchoring titin filaments and stabilizing the sarcomere. TCAP is also involved in T-tubule organization and the regulation of calcium transients, acting as a mechano-electrical link through its interactions with ion channel subunits such as MinK and NaV1.5. Its function is regulated by constitutive phosphorylation at Ser-157 and Ser-161 by kinases like PKD and CaMKII, which is essential for maintaining T-tubule integrity. Clinically, mutations in the TCAP gene are well-established causes of limb-girdle muscular dystrophy type 2G and various forms of cardiomyopathy, including hypertrophic and dilated phenotypes. |
检测原理