Validation Data Gallery
Tested Applications
Recommended dilution
| Application | Dilution |
|---|---|
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
Product Information
60255-1-PBS targets SMN-Exon7 in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.
| Tested Reactivity | human, mouse, rat |
| Host / Isotype | Mouse / IgG1 |
| Class | Monoclonal |
| Type | Antibody |
| Immunogen | SMN-Exon7 fusion protein Ag16615 相同性解析による交差性が予測される生物種 |
| Full Name | survival of motor neuron 1, telomeric |
| 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. |
Background Information
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.