验证数据展示
经过测试的应用
| Positive IF/ICC detected in | HeLa cells, HepG2 cells |
推荐稀释比
| 应用 | 推荐稀释比 |
|---|---|
| Immunofluorescence (IF)/ICC | IF/ICC : 1:50-1:500 |
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
| Sample-dependent, Check data in validation data gallery. | |
产品信息
CL488-86668-2 targets SMN in IF/ICC applications and shows reactivity with human, mouse, rat samples.
| 经测试应用 | IF/ICC Application Description |
| 经测试反应性 | human, mouse, rat |
| 免疫原 |
CatNo: Eg4812 Product name: Recombinant Human SMN protein (rFc Tag) Source: mammalian cells-derived, pHZ-KIsec-C-rFc Tag: C-rFc Domain: 90-151 aa of BC015308 Sequence: QQWKVGDKCSAIWSEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANN 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 | survival of motor neuron 2, centromeric |
| 别名 | C BCD541, Component of gems 1, Gemin 1, Gemin-1, SMN1 |
| 计算分子量 | 30 kDa |
| GenBank蛋白编号 | BC015308 |
| 基因名称 | SMN |
| Gene ID (NCBI) | 6607 |
| 偶联类型 | CoraLite® Plus 488 Fluorescent Dye |
| 最大激发/发射波长 | 493 nm / 522 nm |
| 激发激光 | Blue laser (488 nm) |
| 形式 | Liquid |
| 纯化方式 | Protein A purification |
| UNIPROT ID | Q16637 |
| 储存缓冲液 | PBS with 50% glycerol, 0.05% Proclin300, 0.5% BSA, pH 7.3. |
| 储存条件 | Store at -20°C. Avoid exposure to light. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
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.
实验方案
| Product Specific Protocols | |
|---|---|
| IF protocol for CL Plus 488 SMN antibody CL488-86668-2 | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
