| CatalogCode: | H00006606-A01 |
| ProductName: | SMN1 Antibody |
| Product Description: | Mouse Polyclonal anti-SMN1 |
| Clonality: | Polyclonal |
| Immunogen: | SMN1 (AAH00908, 1 a.a. ~ 283 a.a) full length recombinant protein with GST tag. |
| Epitope: | MAMSSGGSGGGVPEQEDSVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKVGDKCSAIWSEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANNIEQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFLPPPPPMPGPRLGPGKPGLKFNGPPPPPPPPPPHLLSCWLPPFPSGPPIIPPPPPICPDSLD ... |
| Specificity: | SMN1 - survival of motor neuron 1, telomeric |
| CrossReactivity: | Human. Other species not tested. |
| Packaging: | 0.05 ml of mouse antisera with 50% glycerol. No preservative. |
| Uses: | The quality control of this antibody is limited to Western blot on the immunizing protein.
Abnova's recommended working dilutions for western analysis are as follows:
1:500 dilution for ascites
1:1000 for purified Ig
1:500-1:1000 for polysera |
| Background: | This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy; mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7 which is thought to be an exon splice enhancer. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Two transcript variants are produced by this gene. |
| Storage: | Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles. |
| Purity: | Whole antisera |
| Host_Name: | Mouse |
| ListPrice: | 225 |
| AppSummary: | ELISA, WB |
| SpeciesSummary: | Hu |
| ALTnames: | anti-BCD541 antibody, anti-SMA1 antibody, anti-SMA2 antibody, anti-SMA3 antibody, anti-SMA4 antibody, anti-SMN antibody, anti-SMNT antibody, anti-T-BCD541 antibody |
| ProteinTarget: | SMN1 - survival of motor neuron 1, telomeric |
| PackageSize: | 0.05 ml |
| NotesMain: | This antibody is available only in North America and is supplied by Abnova If in stock in Taiwan. delivery is 10-14 days. If not in stock. lead times can be extended. We will inform you of any extended delays within 48 hours. The immunizing protein is also available for this product. Unit cost for the protein is $ 250 (2 ug). $ 305 (10 ug). and $ 445 (25 ug). |
| Gene_Id: | 6606 |
| Gene_Symbol: | SMN1 |
| Omim_ID: | 253300 253400 253550 271150 600354 |
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