验证数据展示
产品信息
60255-1-PBS targets SMN-Exon7 in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.
经测试应用 | WB, IHC, IF/ICC, Indirect ELISA Application Description |
经测试反应性 | human, mouse, rat |
免疫原 | SMN-Exon7 fusion protein Ag16615 种属同源性预测 |
宿主/亚型 | Mouse / IgG1 |
抗体类别 | Monoclonal |
产品类型 | Antibody |
全称 | survival of motor neuron 1, telomeric |
别名 | SMN, SMA, Gemin-1, Gemin 1, Component of gems 1 |
计算分子量 | 294 aa, 32 kDa |
观测分子量 | 40 kDa |
GenBank蛋白编号 | BC062723 |
基因名称 | SMN |
Gene ID (NCBI) | 6606 |
RRID | AB_2881376 |
偶联类型 | Unconjugated |
形式 | Liquid |
纯化方式 | Protein A purification |
UNIPROT ID | Q16637 |
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
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894 ). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA. This antibody, 60255-1-Ig, raised against the C-terminal region (275-294aa) encoded by the exon 7.