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.
| Tested Applications | WB, IHC, IF/ICC, Indirect ELISA Application Description |
| Tested Reactivity | human, mouse, rat |
| Immunogen | SMN-Exon7 fusion protein Ag16615 种属同源性预测 |
| Host / Isotype | Mouse / IgG1 |
| Class | Monoclonal |
| Type | Antibody |
| Full Name | survival of motor neuron 1, telomeric |
| Synonyms | SMN, SMA, Gemin-1, Gemin 1, Component of gems 1 |
| Calculated Molecular Weight | 294 aa, 32 kDa |
| Observed Molecular Weight | 40 kDa |
| GenBank Accession Number | BC062723 |
| Gene Symbol | SMN |
| Gene ID (NCBI) | 6606 |
| RRID | AB_2881376 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Purification Method | Protein A purification |
| UNIPROT ID | Q16637 |
| Storage Buffer | PBS Only |
| Storage Conditions | 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.
