| CatalogCode: | NB100-1936 |
| ProductName: | SMN Antibody |
| Product Description: | Mouse Monoclonal anti-SMN (2B1) |
| Clone: | 2B1 |
| Clonality: | Monoclonal |
| Immunogen: | Recombinant full length protein to human SMN. |
| Specificity: | Detects a band at approximately 35kDa. |
| CrossReactivity: | Cross-reacts with Human, Mouse and Xenopus laevis. Not yet tested in other species. |
| Packaging: | 0.05 ml Mouse ascites. |
| Uses: | ELISA: Use at an assay dependent dilution. IF: Use at an assay dependent dilution. IP: Use at an assay dependent dilution. Not tested in other applications. Optimal dilutions/concentrations should be determined by the end user. |
| Localization: | Cytoplasmic and Nuclear |
| Background: | Spinal muscular atrophy (SMA) is caused by reduced expression or mutations in the Survival of Motor Neurons (SMN) protein. Two SMN proteins (SMN1 and SMN2) are encoded in human chromosome 5. Deletion or mutation in the telomeric copy (SMN1) causes the SMA phenotype. The severity of SMA is in direct correlation with the expression level of the SMN protein either from the SMN1 gene or a different spliced form of SMN from the SMN2 gene.3-4 The SMN protein forms a multi-protein complex with the Gemin proteins. Gemin2, 3, 5 and 7 interact directly with the SMN protein while the interaction of Gemin4 and 6 requires Gemin3 and 7, respectively. The SMN complex interacts with various protein substrates such as Sm and Lsm proteins of the spliceosomal snRNPs, fibrillarin, GAR1, RNA helicase A, the human hnRNP proteins (hnRNPQ, U and R), coilin and p53. Arginine and glycine rich regions in the protein substrates are important for the interaction with the SMN complex. Furthermore, methylation of arginines in these regions by the methylosome (aka PRTM5) enhances their affinity for SMN. The SMN complex is important in various biological processes such as assembly and restructuring of spliceosomal snRNPs, pre mRNA splicing and transcription.5,6 |
| Storage: | Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze thaw cycles. |
| Isotype: | IgG1 |
| Host_Name: | Mouse |
| Buffer: | 0.01 M PBS, pH 7.4 |
| ListPrice: | 285 |
| AppSummary: | ELISA, IF, IP, WB |
| SpeciesSummary: | Hu, Mu, Xp |
| ALTnames: | Anti-BCD 541 antibody; Anti-BCD541 antibody; Anti-Component of gems 1 antibody; Anti-Gemin 1 antibody; Anti-Gemin-1 antibody; Anti-Gemin1 antibody; Anti-SMA 1 antibody; Anti-SMA 2 antibody; Anti-SMA 3 antibody; Anti-SMA 4 antibody; Anti-SMA antibody; Anti-SMA@ antibody Anti-SMA1 antibody; Anti-SMA2 antibody; Anti-SMA3 antibody; Anti-SMA4 antibody; Anti-SMN 1 antibody; Anti-SMN antibody; Anti-SMN-1 antibody; Anti-SMN1 antibody; Anti-SMNT antibody; Anti-Spinal muscular atrophy (Werdnig Hoffmann disease Kugelberg Welander disease) antibody; Anti-Spinal muscular atrophy antibody; Anti-Survival motor neuron 1 protein antibody; Anti-Survival motor neuron protein antibody; Anti-Survival of motor neuron 1 (telomeric) antibody; Anti-survival of motor neuron 1 antibody; Anti-Survival of motor neuron 1 telomeric antibody; Anti-T BCD541 antibody; Anti-T-BCD541 antibody; Anti-Werdnig Hoffmann disease Kugelberg Welander disease antibody |
| ProteinTarget: | SMN (Gemin 1) [2B1] |
| PackageSize: | 0.05 ml |
| GeneralRef: | 1. Liu, Q., et al., EMBO J., 15, 3555-3565 (1996). 2. Charroux, B., et al., J. Cell Biol., 147, 1181-1193 (1999). 3. Paushkin, S., et al., Curr. Opin. Cell Biol., 14, 305-312 (2002). 4. Pellizzoni, L., et al., Proc. Natl. Acad. Sci. USA, 96, 11167-11172 (1999). 5. Baccon, J., et al., J. Biol. Chem., 277, 31957-31962 (2002). 6. Pellizzoni, L., Science, 298, 1775-1779 (2002). |
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