| CatalogCode: | NB100-57859 |
| ProductName: | SMN1 / SMN2 Antibody |
| Product Description: | Goat Polyclonal anti-SMN1 / SMN2 |
| Clonality: | Polyclonal |
| Immunogen: | Synthetic peptide, C-DESENSRSPGNKSDN, representing the internal region of the human sequence according to NP_075012.1; NP_000335.1; NP_075013.1; NP_075014.1; NP_075015.1; NP_059107.1 |
| Epitope: | DESENSRSPGNKSDN |
| CrossReactivity: | Human. Other species not tested. Expected based upon homology: Human |
| Packaging: | 0.1 mg Immunogen affinity purified Goat antisera. |
| Uses: | Peptide ELISA: antibody detection limit dilution 1:1,000.Western Blot: Approx 26kDa band observed in Human Brain (Cerebellum) lysates (calculated MW of 28.5kDa according to NP_075012.1 and NP_075014.1). Recommended concentration: 2-4ug/ml. |
| Background: | SMN1/SMN2 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. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene 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 full length 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. |
| Storage: | Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles. |
| Purity: | Immunogen affinity purified |
| Isotype: | IgG |
| Host_Name: | Goat |
| Buffer: | Tris-saline [ pH7.3] with 0.5% BSA |
| ListPrice: | 295 |
| AppSummary: | ELISA, WB |
| SpeciesSummary: | Hu |
| ALTnames: | anti-SMN1 antibody, anti-survival of motor neuron 1, telomeric antibody, 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, anti-gemin 1 antibody, anti-SMN2 antibody, anti-survival of motor neuron 2, centromeric antibody, anti-BCD541 antibody, anti-C-BCD541 antibody, anti-SMNC antibody, anti-antibody |
| ProteinTarget: | SMN1 / SMN2 |
| PackageSize: | 0.1 mg |
| Homology: | Human |
| GeneralRef: | Setola V, Terao M, Locatelli D, Bassanini S, Garattini E, Battaglia G. Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis.Proc Natl Acad Sci U S A. 2007 Feb 6;104(6):1959-64. Epub 2007 Jan 29. PMID: 17261814 |
| NotesMain: | This antibody is expected to recognise isoforms b and d of SMN1 (NP_075012.1 and NP_000335.1) and all reported isoforms of SMN2 (NP_075013.1, NP_075014.1, NP_075015.1 and NP_059107.1). |
| Gene_Id: | 6606; 6607 |
| Gene_Symbol: | SMN1 / SMN2 |
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