验证数据展示
产品信息
83609-6-PBS targets Dystrophin in IHC, IF-P, FC (Intra), ELISA applications and shows reactivity with human, mouse, rat samples.
经测试应用 | IHC, IF-P, FC (Intra), ELISA Application Description |
经测试反应性 | human, mouse, rat |
免疫原 | Dystrophin fusion protein Ag3408 种属同源性预测 |
宿主/亚型 | Rabbit / IgG |
抗体类别 | Recombinant |
产品类型 | Antibody |
全称 | dystrophin |
别名 | DMD, BMD, 240567C4 |
计算分子量 | 3685 aa, 427 kDa |
GenBank蛋白编号 | BC028720 |
基因名称 | Dystrophin |
Gene ID (NCBI) | 1756 |
偶联类型 | Unconjugated |
形式 | Liquid |
纯化方式 | Protein A purfication |
UNIPROT ID | P11532 |
储存缓冲液 | PBS only , pH 7.3 |
储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
Dystrophin (DMD or BMD) is a large muscle protein whose mutations cause Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), the childhood neuromuscular disorders that result in progressive muscle weakness, respiratory difficulties and cardiovascular dysfunction. Dystrophin is a crucial component of the dystrophin-glycoprotein complex which is essential for muscle membrane integrity and stability. Dystrophin is located on the cytoplasmic face of the sarcolemma and connects the cytoskeletal network to the sarcolemma and extracellular matrix. Multiple isoforms of dystrophin exist due to the alternative splicing, with a wide range of MW (69-72, 110-143, 271, 426 kDa). Most tissues contain transcripts of several isoforms.