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Anti -CUG-Binding Protein 1 Ribonucleoprotein (CUG-BPI, Cytidine Uridine Guanosine Binding Protein 1)
Anti -CUG-Binding Protein 1 Ribonucleoprotein (CUG-BPI, Cytidine Uridine Guanosine Binding
Protein 1)
Pricing
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| Clone |
Host |
Grade |
Applications |
| Monoclonal |
Mouse |
Ascites |
E |
|
| Myotonic dystrophy (MD) is an autosomal dominant neuromuscular disease that is associated with a (CTG)n repeat expansion in the 3í-untranslated region of the myotonin protein kinase (Mt-PK) gene. A (CUG) n oligonucleotides triplet repeat pre-mRNA/mRNA binding protein may play an important role in DM pathogenesis. HeLa cell protein, CUG-BP1, has been purified based upon its ability to bind specifically to (CUG) 8 oligonucleotides in vitro. CUG-BP1 is the major (CUG) 8 n binding activity in normal cells. CUG-BP1 has been identified as isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50. The CUG-BP/hNab50 protein is localized predominantly in the nucleus and is associated with polyadenylated RNAs in vivo. In vitro RNA-binding/photocrosslinking studies demonstrate that CUG-BP/hNab50 binds to RNAs containing the Mt-PK 3í-UTR. The (CUG) n repeat region in Mt-PK mRNA is a binding site for CUG-BP/hNab50 in vivo, and triplet repeat expansion leads to sequestration of this hnRNP on mutant Mt-PK transcripts. | | | Catalog # | C7948-50A | | Applications | Suitable for use in ELISA. Other applications not tested. | | Recommended Dilution | Optimal dilutions to be determined by the researcher. | | Storage and Stability | May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and add glycerol (40-50%). Freeze at -20°C or colder. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. | | CAS Number | n/a | | Clone Type | Monoclonal | | Isotype | IgG1,k | | Clone No | 3C129 | | Host | Mouse | | Source | Human | | Concentration | ~1 mg/ml | | Form | Supplied as ascites, 0.1% sodium azide. | | Purity | Purified by Protein G affinity chromatography. | | Immunogen | CUG-BP1 human nuclear RNA binding protein. | | Specificity | Specific for human CUG-BP1. Species crossreactivity: Human, mouse, rat, bovine, porcine and rabbit. | | | Important Note | This product is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
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