验证数据展示
产品信息
65239-1-PBS targets Polyglutamine in WB, ELISA, Indirect ELISA applications and shows reactivity with n/a samples.
经测试应用 | WB, ELISA, Indirect ELISA Application Description |
经测试反应性 | n/a |
免疫原 | Recombinant protein 种属同源性预测 |
宿主/亚型 | Mouse / IgG2b, kappa |
抗体类别 | Monoclonal |
产品类型 | Antibody |
全称 | Polyglutamine |
别名 | Glutamine repeats, polyQ |
GenBank蛋白编号 | N/A |
基因名称 | |
Gene ID (NCBI) | |
偶联类型 | Unconjugated |
形式 | Liquid |
纯化方式 | Protein A purification |
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
Huntington's disease is a neurodegenerative disorder caused by the expansion of a polyglutamine (polyQ) repeat in the N-terminal portion of huntingtin protein to a length above 35-40 units (PMID: 26047735; 19507258). The mutational expansion of polyglutamine above a critical length causes a toxic gain of function in huntingtin and results in neuronal death. In the course of the disease, expanded huntingtin is proteolyzed, becomes abnormally folded, and accumulates in oligomers, fibrils, and microscopic inclusions (PMID: 25336039). The anti-polyglutamine (polyQ) antibody MW1 specifically binds the polyQ domain of huntingtin exon 1. On western blot, the MW1 clone strongly prefers to bind to the expanded polyQ repeat form of Htt, displaying no detectable binding to normal huntingtin (PMID: 11719267).